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)

A much over due update

This has been an emotionally draining week.  A dear elderly friend fell asleep in the Lord and we attended his funeral this past Thursday.  

On Friday, Mantha had her routine, follow-up cardiology appointment.  At her last appointment things had looked so stable and so well that Dr. S said “see you in 9 months”  WOW!  Two appointments in a row with a “see ya in 9 months”  I was a bit nervous but Daddy seemed to take it stride.

Cardiac wise the last 9 months haven’t been very remarkable (thank you, Lord!) but we’ve had some seizure issues so she’s not been as stable neurologically as she has been in cardiology.

Back to Friday’s appointment.  or back up a couple of days.  I had noticed that her right foot was more swollen than usual.   Sometimes her face would seem puffy but at other times I couldn’t tell.  She didn’t always have “puffy eyes” which is my indicator that she’s retaining fluid.

Daddy took her to the appointment and he brought up my concerns.   The doctor saw them as well and noted that she had gained 7 lbs since her last appointment.  That’s a LOT for her.    She hasn’t had blood drawn as part of a cardiology checkup in years, but  Dr. S has ordered several labs to make sure we’re not missing anything.  He’s also upped one of her meds.

So we hope that there is nothing serious going on but I’d be lying if I said that I wasn’t worried.  She’s been so stable for so long that just having a whisper of something may be wrong is a painful blow to the gut.

Here’s a picture from April 23rd.   We wanted her to take a nap before heading out to our Pascha (Easter) service at Midnight.   She had been making a Pascha card for her Godmother.     The other picture is also from April 23rd.  She was trying on her Pascha dress. 

An update to the latest Seizure Saga

First I just have to type out the date because I think it's just really cool: 09/09/09.

Last week I played telephone tag with 3 of Mantha's doctors. After 3 days, it was determined that, although a marked change in sodium could cause seizures, Mantha's sodium level had not changed and therefor was not causing the seizures.

Her neurologist increased Mantha's medication by .5 cc's twice a day. That's the largest increase in meds she has ever had. We had to watch her over the long weekend and call the neurologist's office on Wednesday 09/09/09 (yes that's today and I had to sneak writing that in there again).

I watched her "like a hawk" and had her sisters and father watching closely as well. The "blankness" and "spacey" episodes stopped. Her vocabulary became complex again. I hadn't even really put together that she wasn't talking as much. She was *engaged* again with us at meals and during her school.

So for now, we stick with the increased medication and see the doctor for a follow up next month.

"No Change"

Sometimes the strangest phrases can mean the best news! I know other moms of cardiac kids understand the wonderfulness of the words "no change".

Today Mantha had her routine follow-up cardiology appointment. She sees the cardiologist every six months. She gets an EKG and an echocardiogram at those appointments and today was no exception. A routine appointment takes about 2 hours.   One thing that was somewhat important but some may think is routine is she pulled off all the 'sticky' leads after they finished the EKG.  It was good therapy!

She *loves* going to the doctor.  Any time we mention going to the doctor, she gets excited and keeps asking every day if it's the day to go to the doctor. She's become and expert at lying still for the EKG and she looks forward to the echocardiogram. That part is easy to understand. She gets to watch a video while the technician does the echo.  The technician commented on how well she was doing being still while she did the echo.

John was with Mantha today. He usually takes her to the cardiologist or we all go. (Last time we all went and made it a field trip. The other girls got see the EKG and the Echo and got to ask questions). John said that Dr. S came in after reviewing all the tests (EKG, echo, and his own examination) shaking his head. The first words out of his mouth were 'I just can't explain it.  When I see the tricuspid insufficiency on the echo, and I listen to her... But she's fine'.  

He can NOT explain why her heart function is so good.  You see, her tricuspid valve leaks. She has tricuspid valve insufficiency. It's side effect, if you will, of the 're-wiring' of her circulation due to her condition.  It's been funny sometimes, when we take her to a non-heart doctor and they listen to her heart.  The insufficiency results in a very loud and pronounced murmur.

Two and a half years ago, Dr. S decided to do a heart catheterization to 'get a baseline' of what her heart was doing.  Before he started the procedure he warned us that the results most likely were not going to be well, that we may even have to look at yet another heart surgery.  When he was done, he started his report of the procedure with 'I can't explain why her heart's functioning so well, but it is.'  

So, by God's grace we are still on our six month schedule of appointments.  Six months is the longest we've gone without seeing the cardiologist.

(Both John and Tess contributed on this post)

Today's Doctor Appointment

We decided it was time to visit the endocrinologist. We haven't seen Dr. G in a few years. That's a great thing! A while back I took Mantha into to our family doctor to get a referral for Dr. G. Mantha is 11 now (12 in August). Our family medical history has females going through puberty around age 11. We wanted to be sure that Mantha is just delayed and that nothing is wrong.

Today was the day of the appointment with Dr. G. John had to take her because I have no voice. We would have preferred both of us taking her but I am thankful that my sweet honeybear was willing and able to do it.

First some GREAT news. Dr. G confirmed that although Mantha is well below the 5 percentile (she's well below the chart period!) she does have a nice growth curve and he seemed pleased with her growth curve. He discussed with John some things that can interfere with starting puberty or cause puberty to be delayed.

Dr. G also confirmed that some previous blood work was all "normal" but did order 4 more blood tests to make sure all was functioning as it should. He also ordered a "bone age" which is a scan or x-ray of the hand to determine how old the body thinks it is.

Surprisingly, we had the results back within 8 hours! Her blood work was all fine and normal, but her bone age appears to be at least three years younger than her chronological age. This news, on the surface, may appear to be bad news but it really is good news! Because her body thinks it is younger than it is, it means she has several more years to grow! Dr. G said this was very good for her height projection. (She will still be quite petite but I was afraid she might not reach 4 ft but she may even reach 4 1/2 feet.)

So she is just developmentally delayed. I can live with that. John can live with that. What does that mean? It means that everything appears to be working the way it should work, but it is taking longer to get there. This shouldn't be surprising at all; this was the child who didn't get her first tooth until she was 19 months old and it was a molar! A molar!! She is so not typical!

And my lesson? I am once again in awe of the miracle of life and God's handiwork. Mantha is fearfully and wonderfully made. Because she is so mentally delayed, it is good that she is physically delayed. God is Good. His timing is perfect.