Spring Follow Up

The follow up cardiology visits are getting easier and easier.  Today Ellie went in for her 6 month check up with Dr. Pinto.  She had a limited echo and was basically motionless while watching Frozen the whole time.  It only took about 15 minutes.  Then Dr. Pinto came in, listened to her heart and checked her pulses, said everything looked good, and we will see her in a year!  I can't believe we have gotten to this point where we only see her once a year.  The pacer checks will still be every 6 months at the hospital with over the phone home checks in between.  Things are looking steady and good.  I feel like it's almost time to shut this blog down.

Another 6 months

Holy Smokes!  I can't believe that it has been a year since I have updated this blog.  Well, good thing, because things have been pretty uneventful. 

Our check ups with Dr. Pinto (Ellie's cardiologist) are up to every 6 months now.  We last visited her a few weeks ago when we also had her pacer checked and an echocardiogram. 

Ellie's pacemaker is doing well, and she is being paced less and less the older she gets.  Time will tell if she will ever be able to grow out of it.  However, this time they didn't make any changes to it but her battery is still on track for replacement in about 3 years or so.

I'm so glad that Ellie is old enough now to stay still for the echo so she doesn't have to be sedated.  She quietly watched Tangled for a quite a while while the tech did her thing.  What a star patient.  It was a pretty comprehensive echo so it too a while, but it showed that Ellie's pulmonary hypertension levels are normal!  That was fantastic news because it is something that she has fought ever since she was born.  It was just as exciting to know that we were able to stop another medication - the $1700/month Adcirca that she had been taking for about a year (thank goodness for the patient assistance program on this one!).  Now that she isn't on any "critical" medications, we find that we struggle to remember to give her her daily amoxicillin.

We are still scheduled to see Dr. Pinto every 6 months for now.  She was almost going to extend it out to yearly visits but because we recently stopped her medication, she wanted to follow up with her in the spring.  Fine with us; we really like Dr. Pinto.

These posts are getting to be less and less as Ellie's life gets less and less exciting in the medical department.  We are so grateful to have this little miracle girl in our family.  She is so full of life and has grown so quickly.  We cherish every day that she is with us.

Looking Good

I guess I forgot to publish this post sometime LAST year...

In the past month Ellie has had 3 doctors visits, all with positive outcomes.

The first visit was to check her pacemaker.  I often forget that she has that pacemaker unless I tickle her on her stomach where there is a hard spot on her left side, or see it when she is stretching.  It has seemed to be the least of our worries over the past couple of years.  At her last pacer check they set her at 100 bpm so her heart would never go below that, but she was capable of beating faster than that on her own.  Now that she has grown bigger, they have decided that her heart doesn't need to be paced that high and they turned it down to 80 bpm which is great news because it means that she can prolong the battery life.  When the battery starts to go bad that is when she would have to have her pacer changed out.  And if she is old enough and big enough at that point they would move the pacer to an area under her clavicle.  According to where she is set right now that should be in about 3 or 4 years.  And at this point it is her next foreseeable surgery.

The second visit was to see Dr. Harnsberger, the pediatric gastroenterologist.  We have always enjoyed each visit with Dr. H because they have always been positive.  She was very pleased with Ellie's weight gain (about 2 pounds since our last visit when her g-tube came out) and she was encouraged about Ellie's progress.  There wasn't a whole lot to talk about.  She released Ellie from her care.  The only thing that we need to worry about GI-wise is the possibility of her getting a small bowel obstruction.  This is going to be a life-ling thing that Ellie has.

Our third visit was for an echocardiogram and an appointment with Dr. Pinto.  Ellie did AMAZING with her echo.  She usually does pretty well, but this time she laid still by herself without me having to lay beside her or hold her, and she didn't need many distractions as she just watched the movie.  I'm glad that she is getting older and that she can understand what she needs to do.  The echo was reviewed and Dr. Pinto came in to talk with us.  I told her Ellie had been off the oxygen at night for a week and we were paranoid that she would get sick in the meantime so we wouldn't be able to do that echo.  We've been watching the kids like hawks and trying to keep the germs away!  Well, we made it and the good news was that Ellie's echo was normal which means that she no longer needs ANY oxygen, not even  at night!  It has been a long time coming and we are so grateful!  It feels like we are normal again.  The only thing that Ellie is on now is a once daily dose of adcirca, a very expensive medication that continues to help control her pulmonary hypertension, and a prophylacatic antibiotic that she takes twice daily.  We have to crush the adcirca pills and hide them in nutella for her to take it.  She doesn't mind taking the medicine every day.  But who wouldn't with a teaspoon of nutella?  Forget a teaspoon of sugar to make the medicine go down!

Last week we had a visit with Ellie's speech therapist, Gaylene.  I had inquired about when she would be done with therapy since she seems like she is doing so well.  We have had early intervention services for a year and a half and they have helped tremendously, but it is also tiring.  Not that I do much extra work with Ellie (she really has done a lot on her own), but they have taught me what to look for and give me ideas of how to help her when she is struggling.  Gaylene did a small evaluation with her and determined that she is on the high end of average for her age.  Next week we will go through a comprehensive evaluation to test her in all areas.  Expressive development was the area that she needed help in the most and the other areas she was pretty much caught up in.  It will be interesting to see where she is at in all categories.  At this point, we feel like she is doing well enough to release her from early intervention services.  YAY!

It has been a great year and we can see the light at the end of the tunnel.  Ellie has come so far with everything and made great progress this year.  We love our precious Ellie Mei (coincidentally, we learned from Rhome that mei mei means little sister in Mandarin.  We named her Mei because it also means beautiful ).

Surgery #? - G-tube closure

Today Ellie went in for surgery to fix this:

 

 

I've lost track of what number surgery this is.  It looks like she has two belly buttons but it is actually her g-tube site that had not closed completely on it's own.  All of the wrinkles are from the tape that we used to hold it together so her stomach wouldn't leak.  The g-tube has been out for 17 days and was still leaking so the next step was to send her to have it surgically closed.  Unfortunately, Dr. Downey, the surgeon who put in the g-tube was right when he said if they are in for over a year they have to be surgically closed.  We had really hoped that it would close on it's own.

 

This was happy Ellie on the way to the hospital. 
She can say a handful of words but can you believe that one of those words is sun-glass-es?
She will say what she is motivated to say!

We had talked to several people about the procedure and could never get a definite answer about what was going to happen.  I still can't believe how different the GI doctors are from the cardiologists with their answers.  The cardiologists always had the same, definite answers and the GI doctors are all over the place.  At first we heard that they would just stitch it up, thinking they would just stitch her on the outside of her belly.  Then we heard that they would use a little local anesthetic and she would be awake for the whole procedure.  Then we heard that they would just use gas to put her out.  When it finally came down to it today, they used the gas to put her out (totally out), then gave her an IV which is how they administered the anesthesia.  So it was like the worse case scenario for us.

 

Ellie in the pre-op clinic.  She was so happy and saying "bye-bye" and "ni-night"
to everyone...and then we ruined her day.

Unlike the last visit to the hospital, Ellie did not mind a thing that they did to her.  She was so happy and outgoing.  They had the pacemaker specialist adjust her pacemaker so that it would make her heart be paced through the whole procedure (Ellie sometimes paces herself).  There was another 2 year old girl sitting by us and Ellie was really outgoing and initiated playing with her.  Then we discovered a sheet of stickers on the seat beside us, almost like someone had left them there just for Ellie.  Oh, how she loves stickers!  She shared the stickers with the little girl and they were all over the place.  She was in such a good mood! The anesthesiologist was Dr. Chen, an Asian doctor who was really nice and personable.  We walked down the hall together and then I handed her over and was expecting a lot of resistance and crying and tears which there were none to be spoken of.  Dr. Chen took her and said "Do you want to come with Auntie Krissy?".  It made me smile because, being Asian, everyone is an "Auntie".  :)

On my way to the waiting room, I passed Dr. Gray who I've named Ellie's hero.  I don't know if he remembers me since it's been over a year but we said hello.  It's fun going back to Primary Children's and seeing those familiar faces.  I always wonder if they remember Ellie since she has had quite a history there.  We also saw the un-conjoined Herrin twins who have been pretty popular in Utah.  This is actually the second time that we've seen them in surgery.  Gosh, if I think Ellie has a history at PCMC, these girls are probably known by their middle names!

The surgery took about an hour and things went well.  The surgeon, Dr. Barnhart, had to actually make an incision (we were told by others that there would be no cuts and they would use a scope...nope), pull the stomach out a little, close it, and then remove some of the scar tissue around the opening.  Dr. Chen said Ellie would be out of "being out" and drinking when I saw her because it was such a short acting anesthetic. 

When I saw Ellie she was sad and it got even worse.  It was hard to know if it was because she was hungry or because of pain.  She kept saying "ni-ni" (milk) so we skipped the clear liquids to see if the milk would calm her.  We tried sippy cups, straws, and bottles, but nothing would calm her down even if it was ni-ni.  The nurse finally decided to give her some pain medication which she said would kick in quickly.  It didn't seem to do a thing.  She was at her max for pain meds so the next option was to give her some phentanol to help her sleep.  It took a few minutes, but she finally calmed down and slept.  Once they moved us to post op we just had to wait an hour since her last dose of pain medication and then we could go home.  When the time was up the nurse took off all the cords and IV and I got her dressed, and then Ellie threw up.  I wasn't sure they were going to discharge us because I thought she had to keep everything down.  We waited another few minutes and she slept the whole time and things were good so we were out of there.

All night Ellie hasn't been able to keep anything down.  She was trying to eat and drink and we hesitated giving her any medication because it was going to be useless.  Then I thought about putting some essential oils on her to help with her nausea.  I pulled out the peppermint and put it on her stomach and behind her ears and I swear that is the reason why she hasn't thrown up.  Essential oils...they really do work!  That, along with some rainy, fresh air, and being out with the neighborhood kids made her get back to her regular self. 

Today was closure for all of us.  Closure of Ellie's g-tube site, being rid of the g-tube and another obstacle overcome. This should be the last surgery that Ellie has until she needs to have her pacemaker replaced in 3-4 years.

Hopefully tomorrow is a better day.

 

 

Happy 2nd Birthday Ellie!



We've had an eventful summer with Ellie's first trip outside of Utah (outside of the country for that matter) and a reunion at a ranch where Ellie discovered her love of ponies.  She does a pretty good imitation of them, you should ask her sometime. Although the look on her face in the pictures is so serious, she actually really liked riding those ponies.  When we were done she would always do her sign for "more".  She was always on the lookout for them while we were at the ranch, and still is.





All of the time spent away from home was difficult to reinforce good eating habits - lots of snacking and some not so healthy snacks.  So I'm excited for school to get started so we can get on a good routine again and handle her eating habits better.  We are still working on getting her to eat more solids, and hopefully wean her off of her bottle soon.  It has become such a great source of comfort for her, especially lately now that she can say "ni-ni" (Chinese for milk), and we give it to her.  In the fall she will probably start an eating class with other kids her age that might help to encourage her.

Ellie has made some small steps developmentally.  She has started to run and jump a little.  And she has started to say most of the words that she used to sign.  Her speech is coming along slowly, but she is still progressing.  She is becoming so much more playful and animated.  So much fun.

 

Two weeks ago Ellie was scheduled for a sedated echocardiogram at Primary Children's in Salt Lake.  It's been over a year since we have had any procedure done there and she had forgotten everything (thankfully).  In order to be sedated, she needed to have an IV and that was probably the most traumatic part of the day.  They took some blood samples while they were at it so luckily she didn't have to get poked twice and soon she was asleep.  The echo took about an hour and the recovery was quick.  We headed back to cardiology to get the results.  We weren't too surprised when Dr. Pinto told us that her pulmonary pressures were still slightly elevated and we hadn't even taken her off the oxygen for any period of time this time because she was fighting a cold.  The cold could have partially been the reason why they were a little high, but also may be because she was on a significantly lower dose of sildenafil than she should be on.  So we go another 4 months with the oxygen at night and evaluate her again.  While we were there, she had her pacer checked.  She is being paced about 80% of the time in the upper chambers and 67% of the time in her lower chambers if I remember correctly.  At this pace, it should allow the battery to last for another 4 or so years.  And hopefully by then Ellie will be big enough that they can move the pacer to just below her clavicle where it will be permanently. So, not too much good news this time.  It was a long and exhausting day.  I can't believe we dealt with so much more for such a long period of time last year.  So glad that is overwith.

We found out that she was not still positive for C-diff, so no changes to her antibiotics needed to be made. We needed a refill on the zinc supplement but the pharmacy we went to flavored it too strongly and Ellie hated taking it. I was afraid it would turn her off of taking her other medications so we stopped it and waited to see what her blood test results would be for her zinc levels. Ellie had become a pro at taking her medications and did so without flinching, without any liquids to wash it down, without a fight at all. It was so nice and one of the requirements to take out her g-tube.

I had asked the opinions of the cardiologist, the pediatrician, and the speech therapist to see if they thought Ellie would be ready to have her g-tube taken out.  It has been a security thing for us and I really wanted to make sure she was ready to have it out before we made the move.  She's been eating, her growth has been consistent, and she has been taking all of her medications orally.  It was time.  We had a hard time getting an appointment to have the GI docs take the tube out.  The first appointment that was available was at the beginning of October.  I pushed to have a sooner appointment because everyone would tell me that we should have it taken out as soon as she could. I could have done it myself but I wanted someone to look at her and tell me what to do.  We finally talked to someone who could get us in earlier and we saw Dr. Harnesberger this past Tuesday.  Ellie was lucky enough to be her very first patient at Primary Children's since she moved practices.  A successful patient she was.  The MA released the water from the tube, took out the tube, placed gauze over it and taped it up.  Unfortunately, we probably could have done a better job ourselves.  She started leaking every time she would cry or put any pressure on her stomach.  It seemed crazy to me that just a couple of small butterfly bandages (which they didn't have) and some gauze would be enough to let the hole close.  We taped it up better when we got home. There is some scar tissue around the site that might prevent it from closing up.  If it is still leaking in a week, she will have to go in and have it stitched up.  We are crossing our fingers that that won't happen because Ellie is fully aware again of who those people are and what they do.  We thought the g-tube would be such a temporary thing (maybe a month or two), but a year and 2 weeks later, while we were thankful for it's use, we were so excited to finally have it out.  That was a pretty good present for her birthday. 

Of course, Ellie had come down with another cold, and the day after having her g-tube removed she started refusing her medications.  When she started taking her medications orally, she has never stepped back.  I started to wonder if we had made a bad decision.  We figured it must have been because she was sick, and gratefully she started taking her medications like a pro again a couple days later.  Whew!

We've spent the past couple of weeks with cousins and Grammy and Grampy.  Ellie has come out of her shell and has been such a smiley, playful, funny girl.  Happy Birthday Ellie-bun!  We love you!

Dr. Harnsberger

Yesterday Ellie and I had our much-anticipated appointment with Dr. Harnsberger, a gastroenterologist who has been highly recommended by many.  We had an appointment scheduled with her for August, but got a call last week to take an appointment on Monday.  I was excited because we would have been waiting for about 3 months to see her when that August appointment rolled around.

This appointment came at a good time since it has been about 6 weeks since Ellie has been eating on her own, albeit mostly milk and only a little bit of solids.  I was curious to know how long they would keep her tube in and what was necessary to take it out.  I had a much better feeling about Dr. Harnsberger than any of the other GI docs we had previously met.  I can see why she is so high in demand.  Coincidentally, she is going to be joining the other doctors at Primary Children's starting in July.  I was excited to hear that because we love having our appointments at the Riverton hospital.

After a thorough health history, here's some of the things we talked about.

• Not only is Dr. Harnsberger a board certified gastroenterologist, she is also a board certified nutritionist.  She recommended letting Ellie try a few different flavors of instant breakfast to fortify her whole milk instead of whip cream.  The instant breakfast will give her the calories, protein, vitamins and minerals that she needs, but the whip cream is fat and she made note of trying to avoid clogging her arteries since she already had heart complications.  We are probably far from that, but it made total sense.  We have tried the instant breakfast a few times and Ellie's not a fan.  We'll keep trying though.
• We are going to re-test her for C-diff.  If she is positive for it once again then she will consult with others to see if there is a better antiobiotic that she should be on other than amoxicillin which could be the cause of the C-diff.
• Long term use of antibiotics might cause a condition called dysgeusia which is zinc deficiency.  Being low in zinc can cause a bad taste in your mouth so you don't want to eat.  We are now treating Ellie with a daily zinc supplement just in case she is zinc deficient.  She said we would see results in about 3 weeks, but she could be on it for a couple months.
• In order to take out the tube, Ellie has to be eating and taking all of her meds on her own for at least 6 weeks.  We have had the eating thing down for 6 weeks already but the medications is what I now dread.  I called to get a new, more concentrated prescription for her amoxicillin yesterday so instead of taking 5 mL twice a day, she will only have to take 2 mL twice a day.  She will also somehow have to down 3 mL of the sildenafil 3 times a day and 1 mL of multivitamins once a day.  And now, add 2.5 mL of zinc to the list.  We have talked about taking her off the prophylactic antibiotic and just treating any fevers or infections as they come.  It may be worth it if the amoxicillin is causing all of these other problems.  And it would be such a joy if, at her next echo, they found that her pulmonary hypertension has completely gone away and we can get rid of the oxygen AND the medication.  Oh, how we wish.  It would take a miracle but it wouldn't be the first.
• I wondered who would take out the tube when the time came.  The doctor who put it in said that if the tubes stay in longer than 6 months then generally they have to surgically remove them, well, more like take out the tube and surgically close up the stoma site.  That is what I have been anticipating.  Dr. Harnsberger was very non-chalant about it and said she could take it out, I could take it out, anyone could.  They would just take out the tube and put a butterfly bandage over it.  If it doesn't heal in 7-10 days then she would have to have it closed surgically.  That was good news.  As of now, the site is still trying to heal, so she has a pretty good chance of it closing on it's own.

It was such a good appointment with questions answered rather than me leaving with more than I came with.  I guess we finally found our GI doctor.

Making Progress

Since we first met with the GI doctors a couple of months ago, Ellie had a heck of a time with her G-tube.  It seems like every time it is messed with things get worse.  After Ellie was on a 3-week round of flagyl to help get rid of her C-diff infection, we went back in to change out her G-tube to a longer one so it wouldn't rub and cause so much irritation at her stoma site.  We experienced lots of bleeding and some stomach content with the new tube, but most of all it was irritating to Ellie and it seemed like there was no answer as to why and how to help it.  It was just her body trying to get used to something new I guess.  After a few weeks and several visits to the GI doctors, we finally went in to see the nurse practitioner at her pediatrician's office and she suggested putting a barrier cream around it so the acid from her stomach content that was leaking wouldn't irritate her skin so much.  With that it finally settled down and things have been good ever since. 

I have not been pleased with the couple of doctors and NP that Ellie was seen by in the GI clinic.  Maybe things are different there, unlike with Cardiology where things are so cut and dry.  It seemed like there were a lot of ho-hums and no real answers.  We have been really happy with Ellie's care from her pediatrician and cardiologist but have not had the same feelings for the GI doctors.  We are going to try another GI doctor in a different clinic that comes highly recommended by everyone.  She is so popular that we have had to wait 3 months to get in to see her.  So by the time our appointment rolls around in August, hopefully Ellie will be ready to part with her G-tube.

Ellie's main struggle this past year has been her lack of appetite.  It has been so frustrating for us (mainly me) to try to feed her and for her to refuse almost everything.  We had hoped with the treatment for the C-diff that her appetite would come back but it didn't.  Everyday we would count every single millilitre of milk that she drank so we could be sure she was getting enough calories in during the day.  It seemed like over the past few months she was only able to take half of what she needed by mouth and the rest we would put through the pump.  I was so stressed out and wanted her to get help so badly that we looked in to some intensive feeding disorder clinics all over the country.  After researching and coming to my senses that Ellie's situation really wasn't that bad, we were referred by our pediatrician to go in for extra speech therapy sessions at the Sandy Rehab clinic.  We have been there a few times.  We play for 30 minutes and then eat for 15-20 minutes.  I think it has been helpful, but it's hard to know since Ellie's progress is so slow.  We are still getting home speech therapy and visits from the developmental specialist.  I don't know if the therapists push me more or Ellie.  All of the time they are telling me to be very silly and animated with Ellie and to do a lot of things that are out of my comfort zone even in my own home, with my own kids.

On Mother's Day we finally got around to giving Ellie a special blessing to help her have the desire to eat better.  It was an answer to our prayers and a miracle that Ellie started eating the next day and we have not had to use her feeding pump ever since.  We have stopped counting her calories and I'm pretty sure she is still gaining weight.  Most of her calories still come from whole milk infused with whip cream and she sometimes struggles to eat much solid food, but she is eating all her calories on her own now which is such a big relief.

Mother's Day
(I wish I would have known my eyes were so squinty)
I couldn't have asked for a better gift than
Ellie starting to eat and being able to go to church together as a family.

A couple of weeks ago Ellie was released from her home health care.  That means no more nurse visits which, lately, were basically just to check her weight once or twice a month.  We liked our home health nurse, Von, but we are happy to be done with her services.

I am often asked if Ellie will have any more surgeries.  At this point, the only thing we know of is that Ellie will have to have surgery to remove her G-tube when the time comes.  I don't know how long they will wait to watch her eat on her own.  We would hate to take it out only to have to put it back in at some later point.  I'm nervous about how she will handle taking her medications orally.  She is scheduled to have a sedated echo at the beginning of August when they will re-evaluate her pulmonary pressures and see if we can take her off her oxygen completely (please, please, please...).  It will have been almost a year to the day since we have been up to Primary Children's in Salt Lake for any procedure.  Wow.

Ellie will be 22 months old on the 17th.  She still has a speech delay but she is progressing.  Yesterday I asked her if she wanted a banana and she will usually sign "want" if she wanted it or push it away if she didn't want it.  Instead we heard her say "nana" for the first time.  Then a few minutes later she wanted to play on the iPad (her favorite toy in the whole world) and she said her version of iPad.  She has been mimicking a lot of what we say and do lately that maybe during this phase she will start talking even more.  In addition to her handful of words a few months ago she has started saying Da-da (which I hear all day long.  It's her favorite word to say), Ma-ma (finally!), done, hi, ow-wee, cheese, and shoes.  It's a good thing that she can sign about a dozen of the essential signs that she needs to communicate with us.

I have been taking Ellie to a parent-tot swimming class while the boys are doing their swim lessons also.  This has been a big social experience for Ellie and she is still quite shy around other people.  She loves the water though, and is good at blowing bubbles (of course when no one is looking) and not afraid to get her head wet.  We've worked on front and back floats, gliding, jumping in to the pool, getting out of the pool, and arm movements.  Half of the time she is just watching, checking out what the other kids are doing with a very serious look on her face.  That's Ellie.

We always wonder what the kids are up to when they are so quiet.
This is what Ellie does - puts headbands around her neck and
reads books in the rocking chair in her room.

We are about to take Ellie on her first venture out of the country.  The only other time we have left home, not including hospital visits, is when we went to St. George a couple months ago.  Ellie did pretty well and the boys were ecstatic to go on a vacation.  They've been a little deprived of vacations the past year and a half.  She isn't a seasoned traveller like the boys used to be, but as long as mom and dad are nearby she is happy.