For better or for worse…

 

Eleven years ago Drew and I made the commitment to spend the rest of our lives together, for better or for worse, in good times and in bad, in sickness and in health.  Today we celebrated our eleven year wedding anniversary here at the hospital, one of  the least romantic places I can imagine spending our anniversary.   Drew bought me a raspberry iced cupcake in the cafeteria that slightly resembled a mini wedding cake the way the icing was swirled on the top.  It’s the thought that counts, and it was also a pretty tasty cupcake if I do say so. 

It  has been a very stressful year for us, as you can imagine, with all of Lucy’s medical issues, but our marriage is strong and we have become even stronger and closer this year than I could have ever imagined.  I love our life!  No, I never imagined eleven years ago today that I would be sitting in the hospital next to our fourth child’s crib side hoping and praying for answers to her health issues.  I did, however,  imagine that we would grow to have the very strong, loving, and connected relationship that we do have that has resulted in the many small children we call ours.  This is our life, and we are committed to making it the best life possible for better or for worse, in good times and in bad, in sickness and in health.  I love you babe!  I know you will probably read this before I see you tomorrow, I’m looking forward to the rest of our lives together…

Now for an update on Lucy, she is doing better, a deep sigh of relief just came from me as I typed those words to you .  I think the saying slow and steady wins the race applies well here.  Her fever is way down, but  ever so slight so they are monitoring her carefully.  Her tube site, or stoma, looks great according to the nurses who have helped me take care of it.  I’ll take their word for it, as a mother it is so strange to see a hole in your baby’s belly that has a tube sticking out of it.  I know I will get over it, but for now it’s still so foreign to me.  Today, I did all of the care and maintenance for Lucy’s tube, thank you very much.  She is still experiencing   a lot of pain at the site,  we are trying to keep her comfortable with IV pain meds.

We started running food through her tube last night, well actually early this morning.  The connection from her pump to her port was put in the wrong port on her tube so it disconnected and they fed the bed instead of Lucy.  She woke at 5 am soaking wet, smelling of that oh too familiar aroma of Elecare.  We have slowly been ramping up her feeds today and weaning her off of her D10 solution.  She has been continuously feed today with the exception of taking an hour off this afternoon when it appeared that she was uncomfortable and stomach bile was leaking into her tube. We currently have her at her maximum feed rate, so far so good.  Lucy is still NPO until we get the swallow study done on Monday.  The  plan is to wait until after her swallow study on Monday afternoon to be discharged.

My mom and the kids came to the hospital this afternoon to play and have dinner with us.  It was so nice to be together as an entire  family tonight.  The kids saw Lucy’s new tube for the first time, they were all relieved to see that she still has her belly button.  I think that they thought the tube was going to come out of her belly button:),  creative thinking.  I would probably think the same thing if I were still a kid.

Drew went home with my mom and the kids tonight.   He needs the rest!   My mom is going home tomorrow, she agreed to stay longer, but we’re pretty sure that we can handle things. We appreciate so much that she comes and takes on the responsibilities of our life for week at a time.  She and the kids have their routine.  In fact Sophie tells me often that Obachan (grandma in Japanese) makes something this way, or does something that way.   According to the kids she makes the best French toast and oatmeal with sprinkles.  She makes me laugh when she tells me how busy and active the kids are and how much they eat, she tells me as if I have no idea.  I know she is exhausted and is looking forward to going home and resting, but she misses them and all their activities and hunger the moment she leaves and goes back to her normal routines.  Thanks mom, for all that you do!

Drew and the kids are going to come back here tomorrow afternoon, we are hoping that they will give Lucy a six hour leave pass so that we can all go out and do something fun in Philly as a family tomorrow to celebrate father’s day.   Lucy and I would love the change of scenery.  It’s weird how quiet and empty the hospital gets on the weekend.  If nothing else, maybe we can go outside and take a nice deep breath of Phily air.  

Thanks for all of your thoughts, prayers, and support, we are truly blessed.

Bedtime update

We are all feeling the effects of the past few days tonight, sleep would be greatly appreciated tonight Lucy girl!  I will quickly update you on what we know.  Lucy is still running a fever, but not as high as it was last night.  She has been on antibiotics for almost 24 hours and I think that they are helping her because her fever is much lower tonight.  I feel like this remaining fever could be a result of her autonomic dysfunction and not her pneumonia.    She is still NPO but we have done two 50ml bolus’ of Pedialyte and so far so good.  She does not like us touching her tube, who can blame her.  Drew and I were trained on the ins and outs of her new tube this evening.  I have to admit that it is a bit overwhelming , but then again so was the NG when we first  started with that and overtime that became second nature to us.  I must also admit that there have been moments in the last few days that I have questioned our decision to put in the PEG-tube, but when I look at her naked, tube-free, adhesive free, smooth skinned baby cheeks I feel better about the whole thing.

I have been pumping every three hours to keep up my milk supply.  Lucy is not able to breastfeed because they are not sure if she is aspirating when she is nursing.  I have many opinions about this and tonight I will keep them to myself, besides I am too tired to go into a tirade.  We are scheduled for a swallow study on Monday afternoon if we are still here.  There has been no talk of being discharged, but that doesn’t mean I won’t put the idea into the doctors’  heads the moment I think that Lucy looks well enough to go home.

Overall she is doing better than she was last night, but she is no where near back to baseline.  Her bowels are moving oh ever so slowly, which makes her dad and I so happy, strange I know.  I know all of you parents out there are just as obsessed as we are about our children's bowel movements.  I think that conversations about our kids bowel movements are  about as common place between parents as conversations about the weather. 

Lucy has also been slow to move around.  She did sit up for the first time since the surgery tonight, but than she just laid back down on her back.  Her tone is very low but improving.  She has slept a lot and we know that is the only way her body is going to replenish its energy levels. 

Lucy is sleeping and so must I, after I pump again.  Thanks for all the support, we love hearing from you. 

Just a few of my favorites…

 

Tomorrow’s another day…

I admit that I set myself up for failure when we originally scheduled this admission.  I was really hoping that we would be discharged today so that we could be home in time for the weekend, which coincidentally happens to be our wedding anniversary and father’s day weekend.  Lucy’s body has other ideas, and right now we need to be here.   Lucy has an aspiration pneumonia that was detected by x-rays done last night, we are treating  her with IV antibiotics.  However, she is still running a fever in spite of the antibiotics and Tylenol.   Personally, I think that Lucy’s body has had enough and is responding to all of the stress with a fever on top of the pneumonia.  She is still very out of it and extremely floppy, when she has been awake she is extremely irritable, wouldn’t you be too! 

Lucy is still NPO, which means nothing by mouth, and now with the aspiration pneumonia they are having us hold off  on breastfeeding even longer.  Lucy has a history of aspiration, we discovered this when we introduced her to water and a Sippy cup several months ago.  Lucy’s suck and swallow are weak due in part to her hypotonia.  She is unable to draw water out of a regular child's non-spill Sippy cup, because of this we tried giving Lucy water through a Sippy cup without the non-spill valve in place.  This is when we noticed that she chokes, gags, choughs, gets red water eyes, and can even sneeze whenever she drinks water.  These are all signs of aspiration so we have been thickening her liquids to a nectar like consistency to prevent her from aspirating which can cause aspiration pneumonia.  Lucy’s speech therapist (ST) said that we could do a swallow study to evaluate the severity, or we could just remedy the problem for now and do a study at a later time when we don’t have as much going on, whenever that is.  In order to start breastfeeding again we need to speak with the ST here at CHOP and discuss the situation.  If I had to guess, I think a swallow study is in our near future. 

Lucy’s NPO status means that we still have not tried out her new hardware.  We have, however, gotten a little bit more familiar with it.  Both Drew and I have discovered that the ports on the tube need to be taped shut or else you will be sporting a very gooey stinky wet spot on your clothing whenever you hold her.  We will be getting a training on all the ins and outs of her new tube (no pun intended) sometime later today.  Lucky for us, her nurse for today happens to be the  g-tube instructor.

We did have to place another IV this morning, frankly the others  just wore out.  D-10 solution and antibiotics cause a lot of wear and tear on IVs.  The same IV team nurses from Wednesday placed her new line and were extra sensitive to Lucy’s situation. Often times a little extra compassion can make all the difference in the world when you are stressed to the max!   Let’s just keep our fingers crossed and hope that this is the last line she will need for this visit!

Great news, we were able to get the results back from the GH torture studies that we did on Wednesday, everything came back normal, yippee!  I hope this puts the whole GH issue to rest.  We have yet to speak with Dr. Langdon directly, but the endocrine fellow came in and gave us the results last night.   Now I feel even better about not doing the last study, it would not have been necessary.

We are waiting to see the speech therapist, waiting on orders to start the Pedialyte, waiting to see what Lucy’s body will do with this fever, and waiting to see her metabolic doctor and geneticist sometime this afternoon.  I’ll update you later…

More Updates

Update at 5:15

 

We are back in our room and Lucy is sleeping in her daddy’s arms. When I hold her I have to hold her standing up because when I sit down she wants me to nurse her, she is seeking comfort, I would be too if I had done to me what Lucy has. She is still NPO and will be until at least 9:00pm tonight.  At 9:00 we will try a slow bolus of Pedialyte  and see how things go from there.  Lucy is pretty out of it  right now, and from what we have been told will be for some time.  They put her under with IV sedation instead of general anesthesia, the drug they used to keep her quiet and still for the procedure apparently has long lasting effects.  The best thing for Lucy is to allow her to sleep and wake up when she is ready.  We are fine with that!

Update 9:00pm

Lucy could use some more thoughts and prayers sent her way.  She has spiked a fever and we don’t know why.  She appears to be in more pain than they typically expect for a baby who has just had a PEG procedure.  She is still very out of it!  We just went down to radiology to get a few x-rays of her belly to see it there is something going on inside of her that could be causing her fever and pain.   Right now there are many scenarios that are being thrown out there for possible causes, we trust that we will get to the bottom of this!  Obviously, we are holding off on any feeds until we know more.  She has been connected to an IV of D10 since 5:00AM.  She has not had anything to eat since than and is not acting hungry, big surprise.  I’ll update later.       

Update at Midnight

The films from Lucy’s belly came back at around 10:30 pm.  The good new is that there doesn’t appear to be any sort of perforation.  Now for the not so good news.  Her bowels are full and dilated indicating little to no movement.   This definitely could be causing her some discomfort, but the high fever not likely.  The films of her belly happened to capture the bottom portion of her lungs which looked cloudy on film.  We did a repeat chest x-ray at 11:15 pm which just confirmed the doctor’s suspicion, Lucy appears to have an aspiration pneumonia.  Antibiotics are on their way up. 

Where is Lucy?

Lucy is currently in surgery having her PEG tube placed.  They are a little behind schedule today, we did not actually get downstairs for her procedure until 12:00pm.  Once we arrived they took Lucy back fairly quickly.  They gave her a little bit of medication in her IV so that when I handed her over to the team it would make the separation easier.  They warned us that kids can start to act sleepy pretty quickly and/or they can act kind of freaky.  Once the meds went in she immediately started giggling and then she scared the life out of us with when a horror movie type scream came out of her.  The nurse took her from me and told us you never know how kids are going to respond.

Update 1:40

Lucy’s now back in recovery sleeping like a baby.   We were told she did well, which apparently they were a little nervous about when they first saw Lucy.  According to the nurses here, they have this theory about red heads, apparently they are difficult to put to sleep.  I can attest to that on a non sedation level but I have no experience with the sedation part.   Lucy handled the procedure well, but her new hardware looks a little scary to me.  I’m sure we’ll get used to it, but for now it’s strange seeing a tube coming out of our baby’s belly, it’s just not natural.

The plan for now is to just let her sleep.  That sounds good to us, she needs it and frankly so do we.  I’ll update later…

So far, so hectic!

I will just begin by saying we are not having an easy time of things.  It all began last night when the resident on call and her med student came in our room to get a detailed medical history on Lucy.   I have come to the conclusion that it is very necessary for me to sit down and write out some sort of current detailed summary of Lucy’s medical history.  I think that it is defiantly in Lucy’s best interest for me to do this.   I know Lucy’s medical history like the back of my hand, but it is exhausting to have to retell it over and over, not to mention very long and detailed for even the best historian to have to retell.  Over an hour later they went into detail about the plans for today.  We were prepared to do the GH lab draw one hour after she fell asleep, and we were prepared to do the three endocrine studies today that coincidentally will take about five and a half hours to complete, but we were not prepared to undergo a twelve hour fasting study.  We were told before we came that a mini fasting study might take place four hours before her endocrine studies this morning, but we were not aware of any plans to do a twelve hour fasting study.   I am getting the impression that as long as we are here they want to do as much as possible,  well we think that it’s too much for Lucy! 

Last night the IV team had a difficult time placing Lucy’s blood drawing IV.   She has one, count it, one good vein in her left hand that has been used over and over for IVs.  It’s what we would call her Ol’ Faithful as that is the only vein the team could find on Lucy’s hands, arms, feet, and ankles to use.   She has scars on her hand and wrist from previous IVs placed there.   I just hate that it is difficult for her to have an IV placed!   I hate even more that I know which vein is good and which veins are not. 

Her IV was finally placed at about 9:30pm and her night feeding began at around 10:15pm because the hospital had a difficult time getting her Elecare ordered, good thing I had some in my diaper bag because the waiting was getting ridiculous.  Drew mixed up her night potion, and they connected her to their feeding pump which alarmed more times last night than we have had with our pump in the last month.   Needless to say, we did not have a very restful nights sleep!

Lucy had her first blood draw at 12:15am which happened to be one hour after she fell asleep.  Well, one hour after that we were finally able to get the blood needed for the first test.  I hope the results are not affected by the variance in time.  At 6:00am Lucy was NPO and that is when our morning began.  Her blood drawing IVs have not been drawing blood,  instead we have had to squeeze the blood from each IV.  It has been a challenging day!

Update at 3:00PM:

We can no longer squeeze any blood from either IV and so we are putting a temporary hold on any more labs until we hear from her endocrinologist Dr. L.

Update at 5:00:

Dr. L just left and we all decided that we are not going to draw any more labs on Lucy tonight! She has had enough for today!  We agreed to come back and have the final study, the CRH stimulation test,  done if in fact we get results back confirming GH deficiency.  We also discussed why and how they would run a fasting study if we agreed to doing it.  I will make a very long story short, we are not going to do it!  Doing a fasting study would mean delaying the PEG procedure a day because GI doesn’t want her on their service immediately after a fast, I think that is very smart of GI.  Our original plan was to have the GH studies done , GI scope, and PEG tube placed, and after much debate we are sticking with our original plan.  I think we all feel better knowing that we are not going to have to stick Lucy any more today!  Tomorrow is a whole other story.

Update at 10:00

The kids came to have dinner with us tonight which really made our day!  Thanks mom for braving the traffic and bringing the kids tonight!

Tomorrow Lucy is scheduled for surgery at 10:00 am.  She will go NPO at 5 am and at that time will get be given and IV of D10, her sugar of choice.   I’ll update more tomorrow, I am tired and am looking forward to getting a little sleep.  Thanks for all your thoughts and prayers today, they helped make a difference!