Post Cath update

Well, we’ve now made it through the heart cath.  After making multiple accommodations (for communicating progress and for her siblings), we attempted to sleep (11 pm when you have a 4:40 wake up just does not work!).

Driving in the dark was generally uneventful (other than the unusual amount of traffic for before 6 am).  We arrived, got checked in, and went up for the prep.  We were both able to talk to doctors and nurses AND answer questions for the intake.  Despite the fact we were a bit late for check in, they were able to take her down on time.  We had to finish some of the intake questions and find the waiting area.  We let our ‘chain’ know she’d started, and grabbed some breakfast (we certainly didn’t miss hospital food!)  

We tried to hurry (but not too much) so we could get back to the waiting area to receive progress reports.  We get back to the waiting area, put our bags and coats down, turn around, and there’s the doctor!  ‘All done’.  Certainly was a shock to us!  We’ve been so used to longer procedures having one completed so quickly was a not unwelcome change.

As far as caths go, the news was good.  Not sure what unit of measure they use for measuring pressures, but they like them to be between 5 and 10.  She’s at a 7.  We think the doctor wasn’t quite trusting what he was seeing, because he told us all the different places he checked:  arms, legs, lungs, (I think the head was the only place he didn’t check).  Since this doctor was not her regular cardiologist, the two need to communicate about the results.

The ‘problem’ with the results are, we don’t have any better idea what is causing the PLE.  The only thing we’ve ‘eliminated’ is high pressures from her Fontan circuit.  We’re still waiting to hear what her team of doctors think is the next step.

Returning to the Unknown: {Yes, it’s an Update}

We haven’t updated this blog in awhile but that is a good thing!  It means things have been going along fairly well for her and she’s been extremely stable. We’ve been able to focus on other areas of our life. Tess has still been blogging  over at Circling Through This Life and you can read there some of the things Mantha and the rest of us have been up to.  Well, in the last two months, that wall (or maybe facade is a better term) has started to break down and we’ve been reminded how very fortunate we’ve been and how very medically complicated she is.

This recent wave of medical complexity began in January, when she saw another cardiologist. (Why do her cardiologists keep moving away? Same practice and this doctor we’ve dealt with before but he’s never been “her” doctor until now.).  We did have some concerns that we brought to the doctor.  She had been extremely tired, more than usual, more lethargic she had no energy. She wasn’t eating as much.  Great news!  Chest x-ray was clear.  EKG was “normal” for her and her echo showed “no changes.”  Doesn’t look like we’re looking at heart failure.  Bad news: Blood pressure  low and lab work came back “worrisome”  with an ‘extremely high’ level of Vitamin D (She has had low vitamin D in the past and one of her doctors prescribed Vitamin D supplements). Dr. M  took her off the supplement, and off her diuretic and blood pressure meds.  She was also very dehydrated.  

In less than a week we had our girl back.  She was full of energy, eating like crazy, and back to her usual contrary argumentative self.  She even willing did and asked to do school. She did not complain about being too tired.  We repeated labs and things were trending in the right direction.  

As the days went by though we noticed that she was beginning to swell.  First her feet and ankles and then up her legs and in hands and face.  We did labs again and the doctor put her back on her diuretic but at a lower dose.  Kidney function had returned to normal. We were told to repeat labs in one month and a doctor appointment was set up.  We’ve got to be out of the woods now right? Wrong.

Her face and  hands went back to normal, and the swelling reduced in her legs.  While the swelling improved in her feet and ankles it did not go away completely.  We called the doctor again.  More labs ordered  and this last go round included a stool sample (the wonderful things we get to do for our kids!)  The results of this last round gave us a major shakeup.  The labs indicated she has Protein Losing Enteropathy (PLE for short).  

This is not an uncommon condition to pop up for someone who has had the Fontan (as she has).  The causes are numerous, so treatment varies according to the cause.  Bottom line though is figure out the cause and treat the cause and that should, in theory, resolve the PLE. Prognosis?  It depends on the severity, and how well it can be managed using the various treatments. You Google ‘PLE after Fontan’ for an idea of the prognosis and treatments (or don’t it isn’t pleasant).  The first step in determining a treatment plan is doing a cardiac catheterization, which we’ve scheduled.  That will lay the groundwork for where we go from here.

A little bit about the cardiac cath:  they will be taking internal pressure measurements and looking for things that may be raising her pressures and causing the PLE.  Things such as occlusions and collaterals.  If they find them, they will try to take care of them right then and there.

It’s been a difficult watching her struggle, while hoping that things are starting to turn around.  Maybe they will, but for us, it feels like the fear and uncertainty we experienced those first few years.  It’s the unknown and that is scary.

Her Cardiac Cath is scheduled for this week.  We’ve set up a Facebook group for friends and family would like to stay updated both for the catherization and going forward.

(Both mom and dad contributed to this post)

New Doctor, but No Changes

 

Gosh, it’s been such a long time since we’ve posted to this blog.  It’s well overdue for an update, so here goes...

About a year ago, we got a letter in the mail.  Mantha’s cardiologist, who she had been seeing for 7 years – since we left the military, was moving to another state.  We had just seen him a month or two before, so we weren’t immediately due for an appointment.  They reassigned her to another Cardiologist in the same practice.  Time and scheduling were such that we didn’t get to  meet the new Cardiologist until yesterday.

The appointment overall went well.  The appointment went quicker than we were accustomed to, as a Cardiology appointment usually takes two hours and we were done in little over an hour.  I’m not sure whether or not that was due to the date (Friday before a holiday) or if it was something else.  The tests run were the same (EKG, echocardiogram) and the cardiologist was nice.  He compared the current tests with the previous appointment results..  The bottom line:  there were no significant changes in her heart function.  We continue with the current treatment plan.  Of course, the spectre of future issues continues to lurk around the edges, but as of right now we continue to be content in ‘no change’.

It’s been a while since we’ve had to ‘break in’ a new Cardiologist.  It wasn’t as difficult as previous times, as this new one was in the same practice as the old one, and I wasn’t having to get records moved and explain so many of the little nuances that make up Mantha’s condition.  It’s also interesting to see what different people focus on when they are treating or discussing treatment.

12 years and counting

Today marks the 12th anniversary of Mantha’s last open heart surgery.  She had the Completion Fontan at St. Christopher’s Hospital in Philadelphia.  It was the third stage surgery in what has been known as the “Norwood”.

She was  2 years 9 months and 6 days old.  She’d already been through the Stage 1 surgery (as well as the the extra trip to the OR four days later for another Open Heart Surgery due to complications) and the Stage 2 surgery.  She’d been through numerous heart catherizations. She’d even undergone emergency surgery for a vascular repair to her subclavian artery due to complications from a heart cath. 

She could not walk.  She had maybe 5 words in her vocabulary.  She had a few signs.  (All done, go, movie and music are the ones I remember most.)  She didn’t eat much.  And I was working closely with doctors and nutritionists to avoid a feeding tube.   She was tiny.  Her 13  1/2 month old sister more than likely weighed more than her. 

A few months after that surgery we moved from the East Coast to the Mid West (Missouri to be exact).   We worked with the local school district so that we could get some physical therapy, occupational therapy, and speech therapy.  They also provided Special Ed (which was closely linked to Speech therapy).   This was provided in our home because of her diminished immune capacity.

Those were discouraging days.  I was told that she would more than likely never walk.  Not even with a walker because she just wouldn’t have the upper body strength.  I was told she would never talk (remember she only had like 5 words *maybe*.  She was pretty non-verbal and only had a few signs).  I was told she wouldn’t be able to communicate using sign language because she lacked the fine motor skills to control her left hand.  We were told to start using a communication board and be prepared to petition for a computerized version.  

Today she will talk your ear off.  I am actually having to train her to NOT chit chat during Liturgy.  She says phrases like “I just don’t care for that.  It’s not my favorite.”

She can walk.  WITHOUT a walker.  Yes she did use a walker and her gait is not the best.  She struggles with balance but she is independently ambulatory.

And yes I already said she could talk but she can also do some sign language.  Oh none of us are fluent in ASL and sometimes she’s not completely able to hold her hand the exact way but she she has a good sized ASL vocabulary.  In fact introducing sign language was great OT for her! 

But she doesn’t just walk and talk now.  Yes, she has cognitive impairments.  She’s still physically as well as mentally developmentally delayed.  But she’s learning how to read and to write and to do math.  She has lots of gaps in her skills.  She puts far to many a’s in her name.  But there was a time when I was afraid that she would never read.  That she would never write.   She likes school. 

I look back over the last 12 years and I am amazed at what she has done. Glory to God for bring us this far.

I’d love to share a photo from the time of her surgery but I don’t have any digital copies and my scanner needs a driver but I did find this one from nearly 3 years after her surgery.   She was using a walker  and on this particular day she had been awarded the “Young Hero’s Award”    Her developmental pediatrician nominated her and he had hoped she would be walking independently.  Later this very weekend she would indeed walk several steps independent for the very first time!

Here she is from Pascha of this year (so just a couple of weeks ago).   She’s much taller but it’s hard to see.  The most obvious difference is the lack of a walker

Just when you thought things were going great

 

     Yeah, it’s about time for one of these.  We thought Mantha was doing well.  She wasn’t having puffiness, seizures under control, making developmental strides, all seemed to be well.  Then, we had her blood drawn after the family strep throat drama. (She was due for some blood work and the strep throat visit for two sisters was a good excuse, I guess to talk about everything else.)

     Results come back, there are a couple of abnormal levels. Vitamin D?  No problem, we start giving her a supplement for that.  Thyroid?  We’ll see the endocrinologist for that one.  It took a while, but we finally got in today.  Yes, the levels were slightly off.  It’s been a while though, so why don’t we get new tests done?  Certainly.  Probably get the results Friday, right?  Couple of days to convince yourself nothing’s wrong.  Guess again, get a call the same afternoon.  Thyroid is more off than before.  So, now we get to give her a thyroid pill daily.  This is not an unheard of issue when dealing with DiGeorge syndrome, so we’ve really been rather fortunate not to have had to deal with this before now.

     There are many times that we start to live in this ‘fantasy world’ where she’s not as sick as she really is, and things are actually going quite well.  You get complacent, and you don’t remember that you are dealing with a very sick girl every day. 

     After the phone call, I went (mentally) through events of the past few months and wondered if I had missed the warning signs, or something else that would have given me a clue.  It doesn’t seem to matter whether it’s something big or small, you do the same thing.  You tear yourself apart in a self blame when you wouldn’t have known anything was wrong 30 or so years ago, and you only know now because of modern medicine.

     That’s our update.  Just another adjustment in the road.  Another issue to read up on and educate ourselves on.  I thought I was done having to study!

 

John

Multiple announcements of good news!

Going to change gears here a little bit, and share some wonderful milestones Mantha has achieved this summer.

1)  Mommy is working with her to start reading and writing.  At this point, she is able to read and write three letter words (cat, dog), knows the sounds of all the letters, but not all their names.

2)  She is able to go to the bathroom on her own when we’re out and about (a sister or parent does go with just in case).

3)  She is now wanting to drop the training pants all together.  She had been stuck on the ‘needing them at night’ for quite a while, but we are finally over that.

4)  She is able to swallow small pills (she’s had liquid or compounded medications, and her diuretic is still liquid).  Her one pill (the most recent change which is her anti-seizure med) needs to be cut down to size, but still she’s able to handle the smaller parts.

On the surface, these may seem to be somewhat mundane, but considering the long road we’re on, any movement forward is excellent!

Quote of the Week

Not sure that we’ve done one of these before, but this one was cute, so we’re sharing it:

     The wife and I were discussing a web post where a man described a wrong number phone call.  The person pretended to be the person the young lady on the other end of the line thought he was (not who she was calling for but a father or brother or roommate, it wasn’t specified).  The man went on to say the person the young lady was calling for was out with another young lady, then making it seem like the young man went out with many different women. 

     The discussion moved on to how our fathers would do something like that, deliberately calling the date by the wrong name to get them in trouble, or saying a different young man or woman called while the date was standing there.  We didn’t realize Mantha was listening in.  After my wife said, ‘it’s definitely something my father would have done.’  She came back with ‘Father John, or Father Joseph?’  She thought ‘father’ meant priest.