Early Intervention

Early Intervention is a state funded program designed to identify children with developmental delays between the ages of birth-3 years. The goal being putting interventions in place before preschool. Muriel had been assessed by them around 7 months and presented a >30% delay in gross motor skills. She received physical therapy for 3 months and at her reevaluatuon she had made great gains and did not qualify. That was last February.
The EI team consisting of a speech pathologist, physical therapist, developmental therapist, and her case manager visited the house on Thursday. They spent 2 hours evaluating her and determined she again has around a 30% delay in gross motor and a 45% (yikes) delay in expressive language. In the area of receptive language she was not presenting delay, nor did she show a cognitive delay! We plan on weekly speech and bimonthly PT as soon as the paperwork goes through.

Insurance vent

Just got off the phone with Aetna. YES, it is 1:59am, YES I have been speaking with them forEVER. In addition to the conversation about the Tracleer medication earlier today, which lasted approximately an hour.
Currently, there is some sort of major disconnect. I am *so* frustrated as to why the Sildenafil medication was filled last month without issue and cannot be done this month. Aetna blames Walgreens for charging too much (over $1,000) and Walgreens says Aetna won't take their override codes...BTW, pretty sure it was in fact the same cost last time it was filled. Bottom line: IT WORKED LAST MONTH---FIGURE IT OUT!!! I told the Aetna rep that we'd be checking into the ICU for nitric oxide in the morning if they wouldn't approve her sildenafil NOW since the Walgreens only compounds at night (and that is how her Rx is written). Finally it was approved for a 15 day supply and I'll be going through all this again in 15 days. Craziness.

I really needed an outlet for that rant and I'm sorry for being so negative.

Happy gal!

Today was a good day!

We visited Dr. VanBergen, Dr. Shetty, and OT/PT today. Her pacemaker is going very well and we have to do at home transmission of data through a land line (who has one of those?!?), but don't have to see Dr. Shetty for 6 months!!! It feels good to be low priority!

Dr.VanBergen said she looked great. Her INR was 2.8, a bit high so we have adjusted her Coumadin dose yet again from 1/2 a pill every third day to every other day. Her liver enzymes were on track so bozintan, her next vasodialating drug has been applied for. That will probably take a month to get to go through....we also got rid of one diuretic, diaril. But the BIG NEWS is that we are trying NO OXYGEN, except at night!!!! So excited. She looks like a different child and seems so glad to not be on a 60' leash! I checked her oxygen saturation when we got home and she was 84-86%!!!
Last, we saw physical therapists that recommended she be reevaluayed by Early Intervention for possible services.

All in all, a great day!!!

INR check 10/24

We went for our first finger stick INR test yesterday, 10/24/13. Her ideal range is between 1.8-2.2, or 2.0-2.5, depending on if you ask the surgeons or the cardiologist :). Thursday it was 4.3. Slightly alarming for me. Again, I emailed Angela who contacted Dr. VanBergen to formulate a plan: broccoli for dinner, no Coumadin Thursday or Friday night, half a dose Sat., etc, recheck INR Monday.

Got the "blues" :(

Last night (10/23) at dinner Muriel was a bit blue. Her feet and nails were dusky. I checked her saturation, which has been around 85 at 1 liter of oxygen, and it was between 80-83. We raised her oxygen to 2, then 2.5 liters and eventually she turned pink and was having satutations as high as 92%! I emailed Dr. VanBergen's wonderful nurse Angela, and she said it is all okay as long as she returned to normal.

Home health nurse

Muriel has come to enjoy her thrice weekly visits from Cherry, our home healthcare nur. :)

Nap time!

Tuesday in the Hospital

I'm sorry I haven't posted but I have been slightly exhausted, both emotionally and physically since Friday. Friday night was very hard because once we did get the fever to 102 range, we were still worried. Of course it was a holiday weekend this past weekend, and of course Jeff went out of town for work on Sunday morning. Saturday Muriel was better, only having a 99.6 fever in the middle of the night, but all day she was fine. I should mention she has not been sleeping well at night, usually up at 11pm for medicine and then 1, 3, and 6am she's been waking up thirsty or vomiting or hot, and has  alot of nasal drainage. Sunday morning Muriel woke up with a barking dry cough and again she was around 99. Jeff left and I took her to the pediatrician. He said it was probably a upper respiratory virus. So I relaxed and went about the rest of the day. Same routine for Monday, seemingly fine during the day, up at night with a low grade fever.

On Tuesday morning, I called our cardiologist, Dr. VanBergen's nurse Angela. Before I could finish telling her what has been going on, she said I needed to come in to the hospital for chest xray and blood work. Luckily, both girls were at preschool and my mom could pick them up at 11:30 and 2:00. It still took me forever to get out the door. Sunday on the way to church, our garage door broke (because Jeff is out of town). So the car with Muriel's rear facing car seat was trapped in the garage. I had to switch around car seats and put one in my mom's car. That sounds like a simple task. I loathe car seat installation, we all have talents and let's just say it is not my forte. That, plus getting oxygen tanks and packing a just-in-case overnight bag delayed departure considerably. We finally arrived at the hospital to the nurse awaiting or arrival. She sent us off to xray and labs. I saw Muriel's xray on the screen as soon as it was taken. I'm not a doctor, but it did not look "clean". I was immediately worried about another plural effusion. Muriel had blood drawn again-10ml!-for the third time in three weeks. Her little arms are bruised again. She had extra blood taken to see if any bacteria grow in a petri dish.

Angela met me in the lab and took us to the ECHO lab. She was wonderful and spent an hour talking with Muriel and I. Dr. Van Bergen looked at the xray and said it was good. I mentioned to Angela that I know I'm not a doctor, but it didn't look that clear to me. Angela said that was just the congestion, and the pediatrician was right, that it was just a virus. All her blood (white/red) were in normal range after just having surgeries. I asked her why when she had 103 fever was she having oxygen saturation all the way at 94, and Angela explained that the body's reaction when we have a fever is to have a higher cardiac output, thus more oxygen in her blood. Dr. VanBergen was in the PSHU yesterday, but he didn't come downstairs to see us, which made me happy, since he was helping truly sick kids! Dr. Ross did come to take a look at her and sent us on our way.

At any rate, the final consensus is Muriel is a-ok. Now we just need to get rid of the fever, which was 99.1 this morning. Too much tylenol does not help her liver congestion. I told Angela I'm not coming back to the hospital again until INR testing the 21st, and then the doctor on November 7th. :)

Worst. Night. Ever.

Thursday evening while I was tutoring Jeff fed the girls dinner and Muriel threw up her dinner and then twice more that evening. No fever. We looked at her records and it had been 3 days since her last BM.  I blamed myself for not being more dilligent with giving het miralax. I think I thought of constipation as being a temporary problem, but with Muriel's diuretic regime, it seems to be an ongoing issue. Thurday night we thought she was constipated. Friday at 5am she had a low grade fever 99.6. We gave her Tylenol. Through the morning she didn't eat much, and we gave her more miralax and two supositories. By 1pm she again had a 99.9 temp. I gave more Tylenol. At 3:45 our home health nurse came and again she had a low grade temp. She advised we give Tylenol again at 4, and to call the doctors Saturday if she didn't kick the fever by then. I was hoping the fever was due to teething, since she's got a few coming in.

Of course Firiday I went out for dinner with my friend Kristi. Jeff texted me she had 102.5 fever. I rushed home. By 9:30 it was 103.5. We called the doctor and he told us to come in if we couldn't get the fever down within an hour. Well we did get it down, but it has been difficult to shake completely.

It is now Tuesday and I'm sitting with Muriel in the hospital waiting for a chest xray.

Hospital Visit Today

We spent 4 hours at the hospital today for a clinic visit with Dr. VanBergen. We had blood drawn and a chest xray first. Her labs came back great! Her sodium was 139, an all time high. We are cutting back on one of the sodium suppliment doses as a result. Her potassium was 3.4, up from 2.5 last week. I attributed the rise in both electrolytes to her all Gatorade liquid intake. She may have cavities by 2, but the Gatorade has potassium and sodium! Her chest xray was beautiful.

Dr. Shetty and her nurse Janise brought their pacemaker gear to the party and she told us she is looking good and she will adjust her pacemaker in a month in order to save battery life and put off the next surgery for as long as possible. Muriel did well with that test thanks to nurse Angela blowing bubbles to distract!

By the time she was up for her EKG and Echo, Muriel was not happy. She cried her way through the echo and finally fell asleep at the end. Then the echo tech redid most of the shots since she was calm.

Dr.VanBergen said everything was good. She is not however getting rid of the lasix or diuril diuretics for another 3 months. Aditionally, we shouldn't expect to get rid of the oxygen for another 2-3 months. This news is more than slightly disappointing. I had thought she would be off oxygen in 2-3 weeks, not months. I suppose Muriel is healing at her own pace. We also learned she will have her fenestration closed in around 6 months. That will only be an overnight trip to the hospital. Our next appoinent isn't for a month, with one trio to check her INR level in two weeks.

Movin and groovin

Aunt Kate left today after an entire month away from home.

Muriel enjoyed the park today!

Hospital Visit

Muriel had a fantastic trip to the hospital!!! She has not lost too much weight and NP Carrie said it was to be expected seeing as she is on all the diuretics and the fat free diet. We were hoping to get rid of one of the diuretics, diuril, and only have lasix, but only one dose of diurul was removed from her medicinal regime. She had blood drawn from a fantastic phlebotomist, one needle stick was all it took. From the blood work, we learned her kidneys showed she was dehydrated (expected) and Muriel's potassium was low. Carrie spoke with Dr. Sajan and considered adding another supplement for her, but instead upped the aldactone and suggested bananas and kiwi. Muriel isn't loving bananas as of late, which is a bummer since she used to eat 2 for breakfast every morning. We bought kiwis and gave her a bite; she actually liked it! Then I thought, "Hmm, better check those for vitamin K" (interferes with her Coumadin blood thinner). Of course, kiwi has vitamin K in it, so those are out. My new solution for sodium and potassium: Gatorade. No more water, only Gatorade! It's got both, so hopefully this helps. Her chest xray showed no fluid build up and Carrie was as relieved as we were. After I called Monday to report her low saturation levels, Carrie said she expected to see fluid, but no! We see Dr. VanBergen next Thursday and hopefully he will give us more good news.

 

I'm sorry for not posting sooner. I have been spending 2-3 hours a day on the phone with the insurance company trying to get them to approve medications and equipment. We have had to pay out of pocket for the Viagra, which is a very costly drug! On several occasions, we have joked that Jeff should go to the doctor and get a prescription! We have had wonderful support from Dr. Ilbawi's nurse practitioners Jane and Carrie. Dr. Imran has joined the fight and himself called the insurance company. This irks me because those people have far better things to do, like saving childrens' lives!!!

 

On Wednesday, we went for a walk with our O2 to go and Muriel feel asleep. We took a pic of her without her oxygen when we replaced the tegaderm and duaderm and nasal cannula. Muriel continues to get stronger every day. She has started to use a baby walker to get around and occasionally takes a few steps on her own. It has been a joy to hear her laugh again. She celebrated her 18 month birthday on Sept. 29th. Time flies!

Challenges

It is certainly a great deal easier to post the trips outside and smiles than some of the other challenges.

Muriel has a home health nurse that comes three times a week. She was here last Thursday and Friday. When she came today, Muriel had lost weight. It is a challenge to get her to gain back those lbs when she is on a strict 0 fat, no vitamin K, and egg free diet. She is not interested in eating too much and likes water more than juice or skim milk. She is drinking a bit more and almost meeting her fluid restriction of 730 ml a day on some days.

More concerning was her oxygen saturation today. The goal is 85 and when the nurse came today, she was in the low 70's. I turned up the oxygen to 2 liters from one and she came around to the low 80's after about 20 minutes. I kept her at 2 liters all day. Around 3:00 I checked again and she was in the 70's again. I turned her O2 up to 3.5 liters and she hovered around 80. I called the hospital and talked to Jane, a nurse practitioner. She was concerned but asked how she looked. I told her she looked fine. Not blue and playing. She's not eating, has low sats, can't walk, and vomits some meds, but she looks good! Jane called Dr. Sajan and he said he's still concerned about another plural effusion, but if she looks okay, we can wait until tomorrow to see her. We see Jane at 12:45 tomorrow, so lots of positve thoughts and prayers, please.

The Hunchback of Naperville

Outside!

We went to the park today. We had the place to ourselves and Muriel loved it! Madeline stayed home, as she had a stomach bug and woke up vomiting at 6am. We have had her in quarantine and she ate two meals for dinner tonight...I think she's better now. ;)

Super Muriel run

Some of our humongous support system ran/walked to support Muriel this week in the Plainfield 5K. YOU ARE AMAZING, thank you!!!

Suuuper Muriel

Thank you to everyone participating (and the over achieving runners :)) in the Plainfield 5k on Sunday to show your support for Muriel!!! We loooove our Super Muriel shirts and can't wait to see pictures of all of you wearing them! Jeff will see you there. You are truly the kindest, most thoughtful friends we could ask for. Thank you, thank you, thank you!!!

Honk!

Home at last!

After 25 days in the hospital, we arrived home last night.

Home!

What a very long day.  Its Wednesday (well technically Thursday morning) - a day earlier than Dr. Sajan expected us to leave as of this past weekend.  I was gone all day Tuesday and Dr. Nater actually wanted to discharge us then.  We appealed for one more night - mainly so I could be there to help get her ready and everything packed up, but also because we are both still a little unsure about her oxygen saturation levels.

She is still on 1Lpm of oxygen, which really isn't much, but without it she drops pretty low - into the 70s.  The target according to Dr. Sajan is 85%.  So we went home with an oxygen tank.  On arrival we found that the oxygen company had delivered a whole room full of tanks and a large machine that somehow produces close to 100% oxygen flow from air, with a 50 foot nylon (?) tube.  I'm dying to understand how that works.

We are also squeamish about her weight.  When we arrived a month ago she weighed close to 10Kg.  On checkout she weighed 8.2Kg.  Close to a 20% weight loss!  Wouldn't mind that myself, but on her it makes her look like skin and bones.  We know she is on heavy diuertics, and we can blame most of it on water loss, but also that she hasn't been eating well.  Then the vomiting of her meds and anything she did manage to eat.  We were assured that all of these things would suddenly, magically, appear to resolve themselves when we get her out of the hospital atmosphere.

I'm happy to say that it already seems to be the case.  I made some of her favorite (fat, vitamin K, and egg free) pasta and tomato sauce, and she ate like a horse tonight.  She crawled around on the floor after her sisters - tethered by the 50ft tubing - and seemed to be in a state of shock that she was home.  Megan's phone is still trying to upload the pics - perhaps will be online sometime tonight or tomorrow.

I'm off to bed, but will post more tomorrow about our insurance nightmare.  Should serve as a warning to any Fontan candidate parents following our story.  I'll include her medication schedule which we have to manage.

VERY happy to be home, and can report that Muriel is sleeping soundly in her own bed.  She still has a pulse oximeter tied to her toe, a nasal cannulus for the oxygen, and an Ng tube for meds, but compared to the multitude of things hanging off of her in the past few weeks, she must be relieved.

Outside!

We were outside for about an hour yesterday afternoon. Muriel soaked up some rays and even stood and attempted to walk (with assistance)!

Oxygen

Looks like we are going home Tuesday or Wednesday of this week! We will be dragging an oxygen tank with us though. Unfortunately Muriel is not able to keep her oxygen saturation up without oxygen support. So she will be hauling a giant tank around, or rather I will be following her around with it!

Boo-boo

Muriel's wrist from the IV... :(  It is out now. All that remains is her PICC line and her arterial line.

Officially three weeks in the hospital

Three weeks ago today we drove Muriel to the hospital at Dr. Shetty's orders.  It seems more like three months.  But we are nearing the end.

High fashion

Yesterday her left chest tube was taken out, and the x-ray this morning showed no fluid.  Thank goodness!  Today the IV in her right arm was taken out, so she has use of both hands again.  Her nitric oxide (a mix in her oxygen to keep her blood vessels dilated) has been slowly weened over the past 24 hours, and sometime tonight should be gone.  A giant machine is involved in the NO mix, which has kept her from being able to take any more wagon rides.  She has been on milrinone since Tuesday for the same reason - to dilate blood vessels - which has kept her from going downstairs to the main floor (not sure why they don't want you to be on the main floor on this drug, but that is my understanding).  We have been slowly weening the milrinone as well, and expect to be off of it by tomorrow.

She has been getting IV heparin to anti-coagulate her blood (to avoid the blood clot issue we discovered in her artificial fontan vessel).  That will be replaced by cumadin, which we will be going home on, presumably for life (or until they can find something better).  She got her second dose of cumadin today and have been weening off the heparin.  Expect to be off heparin tomorrow.

She still has the arterial line in her leg and the double access line in her left arm, and the Ng tube for giving oral meds (that has made a world of difference in her vomiting, and as a result her sodium levels are now normal).  Oral meds are still fairly plentiful - some diuretics (which will continue at home for a few months), some to protect her stomach and intestines (saw some blood in her vomit, and suspect she is having stomach troubles), various electrolyte supplements.  All are needed less and less though, as we get closer to getting out of here.

Sleeping on mommy's lap

As for her clinical state, she is almost back to the little girl we brought in here three weeks ago.  She gives the doctors dirty looks, throws her toys off the bed, and is able to point and yell for the things she wants.  She *really* wants out of the bed, and was even standing for a few minutes last night (a bit shaky though).

Its starting to look like Tuesday could be the day we get discharged, which is good and bad.  As Megan mentioned my grandfather passed away this week, and the funeral is Tuesday.  I have family flying in today and tomorrow - will be a very busy week.  Megan may have to get packed up without me here, though either her mother or sister will likely be able to help.

We should know more tomorrow as meds stop and we see how she does.  It is just as likely that they will want to keep her a day or so more.  We want to desperately avoid having to bring her back here, which I understand is common for children completing the Fontan.  Hoping our "return" visit was built into this extended stay already.

Will keep posting even after we get home, as I am hoping this will serve as a log for before, during, and after our Fontan experience, such that other parents going through this know what they might expect to go through.

Happy girl!

Chest tube out! Versed makes her happy :).

Turned the corner and headed down the street!

Muriel's blood cultures are NOT growing! We won't have a definite negative for 5 days, but as Dr. Penk told us last night, the results they get "excited" about come back relatively quickly and are treated agressively. If she does have a positive result in the next few days, they will simply administer antibiotics. In summation, no blood infection so far!

Muriel hasn't been interested in food this morning and what we did get in-3 grapes- she gagged but kept down. This could be due to the NG tube. Things are definitely going well and she is getting her last chest tube out today! Coumadin will also begin again tonight, which will take around 3 days to get the dose correct. This means we could be going home as soon as Tuesday! Sadly, Jeff lost his grandpa and the funeral is also on Tuesday in Polo, IL.

New toy!

Muriel and daddy put a fun new toy together today. The child life department here at Hope is phenomenal and generous!

NG for me :(

After vomiting countless times, Muriel got a feeding tube inserted today. I am actually glad. Watching her vomit after every medication- especially sodium supplements- is heart wrenching. We are used to the NG tube, as she had it from birth to 4 months. It (hopefully) won't be coming home with us.

Thank Goodness for Insurance!

So thankful for insurance. Muriel's trip to the cardiac Cath lab on August 20th was billed as $38,000. This does not include the doctor bills from the cardiologist or anesthesiologist or the stay in the hospital after! 

Fever

Last night around 10 pm Muriel spiked a 102.6 fever. Dr. VanBergen was on and ordered a blood culture. She needed a sample out of her PICC line and out of her blood stream. This meant another blood draw and needle prick. The arterial line and other IV couldn't be used since there is a chance that an infection could be in the lines, not in her blood. The blood was sent to the lab to see if any bacteria grow in a petri dish. We were with her until 3am when we finally needed to sleep. When we arrived at 9am, she was just waking up and again had a fever, just 100.8. She vomited all her 9 am meds. She had a catheter to extract some urine in case the fevers are due to a UTI (doubtful). With the catheter she got Ativan her anti anxiety drug. With that came some cooing and smiling, much to mom's delight. Dr.Sajan also heard her and came to listen and laugh along. Grandma and Aunt Kate are here now to visit and Muriel is taking her afternoon nap. So far, no goobers in the petri dish!