Thursday, April 29, 2010

Wisdom

Oh My Sweet Baby.

She has slept really well through the night. So well, that I am having difficulty getting her to finish her bottle. She sleeps like a little angel and I hate to wake her up, but I have to... every 2 hrs. It stinks because we are putting rice cereal in her bottle. This is to help thicken it up, so that it has a harder time coming back up. Unfortunatly, it makes it thicker, so its harder for her 7lb body to suck it out. Also, it has to dissolve really well, so you have to get the bottle really hot. So here is an outline of a typical feeding now:

8:45am- Cup of water goes into microwave for 4 min. I get the bottle ready (pouring formula and adding rice cereal, put bottle together) figure out what meds she needs to take, get that in a syringe. 8:50am- put the ready bottle into hot water. 8:55am pick baby up to begin waking her, change diaper. 9:00-baby cries, check to see if bottle is ready. 9:30am bottle is done or as done as its going to be. Calm baby down, try to get her to go back to sleep. 9:45 Mom crawls back into couch that has become her bed. 10:45 am, process begins again.

My cognitive thinking skills are beginning to get fuzzy. I am not trying to complain, but I get somewhat cranky when I can't sleep. I am hoping she can outgrow this reflux mess very soon. Its so hard. For me, for Patrick for sure, and all my friends who keep hearing the same things over and over again. I am very thankful that they dont hang up the phone or ignore my texts. I would be utterly lost. I keep praying, but I don't hear anything. I know He has not left me. I know he is with me, helping me day to day, and I could not imagine going through this without God by my side. He was with Job, when he was being tested, and my situation is nothing like that. I just keep asking him for strength and for the wisdom to do what is right for Evie. Sometimes I do feel alone. I wonder if I am just not listening well enough. I know this happened to us for a reason. I can take care of her, unlike some other families out there. God gives special Babies to special families, right? These are just the things I tell myself, to get through the day (and night ha! )

God has given me some wonderful family and friends. They listen, they help out when they can and most of all they pray. I am so thankful for you guys. Thanks for not giving up on us or getting annoyed with us! We love you!

Monday, April 26, 2010

My Mistake

So Evie is doing much better. After talking with a good friend this morning, I found out that by actually looking at the nipple, on the rim, you can find out its size. Well, as it turns out, 2 of the nipples were level 2s! Poor thing! I wish that I had known to even look for that! So my Mom went out today and bought some newborn level one nipples. Uhhg. If only I'd thought of that, we probably would have been spared all this extra grief!

To let you know, her Special Formula is called Portagen. It is made with medium chain fats. These are the kinds of fats she can have. AS far as her formula goes, I make it up every 24hrs. I make enough to last the entire time period. Her formulation can change every week depending on her weight and how many calories she is supposed to be having. Right now, her formula consists of Portagen powder, water, canola oil (this is another source of Med. Chain fats that help to sustain her, and 1 oz. of breastmilk. The Breast milk will never increase. She gets 1oz a day, until I run out. After we found out about this, I could not breast feed her due to all the long chain fats found in Breastmilk. I did, however, pump enough to last a good while. I stopped because not only was it incredibly painful to only pump, but my supply had dwindled down to hardly nothing. I tried More Milk Plus, I tried hot showers before, I tried relaxing and praying before I would pump, and still, I got about 10ccs. her formula provides all the added nutrients she would usually get through breastmilk or even other formulas. As far as I know, it is not lactose free. As a matter of fact, she will probably have to drink this to have those nutrients well into adult hood because her body needs vitamins and things that she will be missing out on because of her diet. Most LCHAD Children drink this mixture Morning, Noon, and then at bedtime with added cornstarch to help it last longer so that they don't have to wake up as frequently to eat. Often, they have to drink this either before or after exercising, sometimes both, because they use up a lot of energy and this is the best way to get them more.

Every other day she gets a dose of DHA, for her eyes. This supplement helps to break down fats behind her eyes so that they stay in good shape. There is concern for LCHAD patients to develop retinopathy, a disease of the eye. Taking DHA is a way to prevent this.

I hope I have answered all questions reguarding Evie's eating habits. Please feel free to ask more! It is a learning Process for me and my hubby. We are always striving to learn better how to prevent a metabolic crisis.

Sometimes I want to cry

So at approxamitly 8:30 this morning, after having gotten up every 1.5 hrs through the night, my sweet baby threw up. Thats right. I did everything the Drs have told me to do. I give her meds with almost every feeding. I feed her every 2 hrs. I check her blood sugar as needed. Everything. Not only is she running out of clothes and blankets, I am too! Thankfully this time, she was in her swing, and it didn't get on me. Not that I don't love the smell of regurgitated formula. Its a smell only a mother could love.

Seriously, I am going to try one more "trick" so to speak. We were given some Dr Browns Bottles, that were gently used. Now something I had not thought about is maybe the nipple on the bottle isn't for newborns. We were given a whole bag of them big ones and small ones, so who's to say maybe the nipples didn't get mixed up. My mom is going to burlington today to see if they have newborn nipples. I don't have to use these. I will be looking into other bottles, ones that maybe help with reflux. At this point, I am not even sure if I should call the Dr. Its kind of like old hat to us.

This time was a bit different. We had just finished eating and burping. She looked sleepy and I had dishes to do so I set her in the swing. I came back after about 15 min and there it was. Lying there all over her, her blanket, her socks that had, of course, come off, and over her swing. So, at first I started to cry. Then I picked her up, changed her into one of 2 clean outfits, then went to the laundry room and put all of her clothes in the washing machine into the dryer. So that if it happens again in 30 min, I will at least not have to have a naked baby.

So, not only did she throw up this morning, but I am afraid my dishwasher is on the fritz. I have run the same load, moving thigns around 3 times now and they are still dirty. Lovely.

My goodness do I love her. I think that is why I get so upset when this happens.. I want to see her smiling with her milk moustache that she always seems to have. I want to see her with her tongue sticking out with probably days worth of formula on her tongue that I can't get off. Lastly, I know that if I don't feed her every thirty min. now, she could possibly have to go back where she will be poked and prodded. Uhgg. Sometimes I do want to cry.

Friday, April 23, 2010

Wow. Can I do this?

Ok, so after last night's little mishap with Evie projectile vomiting, I obviously was concerned. We did everything right as far as adjusting her feedings so that she got little bits throughout the night. Thankfully my Mom came over and helped ALL NIGHT! Whew! I was actually able to get some sleep. I function so much better when I sleep even a little bit. This was the first time in a long time that I got 5 hrs of almost consecutive sleep. I would wake up and check on her then go right back to sleep! Mom was a big help!!! I called her Ped. first thing this morning, and of course he had to call back, but he did pretty well. We weren't in a crisis so I wasn't too concerned, just so he called before going home for the weekend. I asked him what he thought we should do and we both came to the same consensus. That I should feed her every 2 hrs day and night. Now upon first hearing this, it doesn't sound too bad. After all, breastfed babies are usually fed at 1 1/2 to 2 hours at a time so whats the big deal? Well, we cannot let her sleep. Usually BF babies can sleep up to 4 hrs if they pretty much choose to. With Evie and LCHAD, she has to have a constant supply of food so that her Blood Sugar doesn't drop. I am hoping we can do this for a couple of weeks, then go back to the 3 hr feedings. Hopefully thats all it will take for her to grow out of it. I sure am going to miss sleeping!! Forgive me if I become cranky. Things may be a little hard.

Thursday, April 22, 2010

Shes at it again

Evie vomited. Again. I sure do hope her meds start kicking in soon. So, for the remainder of the evening, I am going to give her 10cc every 30 min till we get to 2:00am. This will be how much she is supposed to have in a 3 hr period. At 2:00 am I will give her 20ccs every hour till we get to 5 am. At 5 I will administer 30 ccs, I will repeat at 6:30. Then at 8 am she will get 40ccs every 2 hrs, and so forth for the rest of the day. I am worried, because when LCHAD babies do this, sometimes they will insert a feeding tube in either their throats or their bellies (G-tube) I will call her Ped. in the morning relaying this info. Thankfully my mom lives only about 15 min away and she is coming over to help with the feedings tonight. Unfortunately this means that neither Me, My mom, nor Evie are going to sleep tonight. I told Patrick to go ahead and go to bed. I am going to need him tomorrow night!

Birth and Diagnosis

Evie was born on Feb 6, 2010. She was almost 8 weeks early. I went in on Thursday Feb 4 for a routine office visit. I remember I was excited because I was supposed to get our last ultrasound done and I was anxious to find out if she was really a girl!!

I went in to the US room and they told me she was slightly underweight. It shouldn't be a big deal, we would just have to watch it. I then walked down the hall, to an exam room, and if you have had a baby, you know, they always check your Blood Pressure. Well, I knew something was wrong when the nurse took it, the said well, I'll be right back. She came back 5 min later and took it again. I don't think there was any change cause she said hang on I'm going to ge the Dr. So at this point in the visit, I was pretty nervous. Dr Tynes walked in checked it, then proceeded to have about 3 other people check it with different cuffs. It was then when she told me that I was not going back to work, but rather I was going to be admitted into the hospital for several tests. She was hoping I would be on bedrest for the remainder of the pregnancy. She consulted with another Dr, who told me I would probably have to deliever on Sat! Apparantly I suffered from the HELLP syndrome (common in LCHAD babies mothers). My liver enzymes were high, I was on the verge of renal failure, not to mention the hypertension (high blood pressure). Essentially, Evie could not grow anymore, and I could not survive with her still inside me. We were killing each other.

They gave me 2 days to keep her cooking. They gave me 2 shots of steroids in hopes that her lungs would develop more quickly. Saturday came and sure enough they wheeled me into the OR, where she was born at 8:16 am, 3lbs 5oz, 16 1/2 in long. We were told that she could possibly have Downs Syndrome, so my first question was "is she ok?" Of course she was perfect.

She stayed in the NICU for 5 weeks. It was here after about 2 weeks, that the results of the most important test in her little life, came back elevated for LCHAD. This is a fatty oxidation disorder, meaning she has problems digesting certain fats. Hers are long chain fats. Long Chain fats consist of most oils, butters, anything fried, etc. See, we all carry the gene for LCHAD. This specific gene helps in the digestion/absorption of fats. Hers is either mutated, or simply not there at all.

She will be on a specialized diet for the rest of her life. I was concerned because for 2 weeks we were trying to breastfeed and it was not going the best. My milk took FOREVER to come in and when it did, I was not getting enough to sustain her. I was worried though because I knew breastmilk for an LCHAD baby was not good. The fat could build up around her heart and eyes. We quickly changed formulas to one that is special and has to be ordered, Portagen. She is still on this and every week or so I have to call a Dietitian to give her Evie's weight and then we change the recipe according to how much she weighs.

When she gets older, we will be able to give her more fats, but still only about 5-10 grams a day. If you look at fat labels, this amount is not much. This includes everything she will eat in the course of the day.

I hope this helps you to understand the significance for Newborn Screening. The Drs say, that SIDS is ultimately the cause for undiagnosed FOD's.

There is a wonderful website you can look at that has several different Fatty Oxidation Disorders (FOD's) www.fodsupport.org This is a great resource for learning to deal with FOD's on a daily basis. They provide recipes and support for families living with FODs all over the world.

Our First Visit to the Hospital

Evangeline began vomiting at 9:00, last Friday morning. In case you may not know, there is much significance when an LCHAD baby vomits. Since she cannot process Long Chain Fats, she She needs to eat frequently to keep her Blood Sugar high enough so that she doesn't enter into a hypoglycemic state (which can be very dangerous for any baby; causing developmental delays, mental retardation, and much more. Although it does have to be extremely low in order for that to happen)

At this point, I wasn't too worried. She had spat up before and usually her Blood sugar was at a relatively good point, at least until the next feeding. Well, at this time, we were on a feeding schedule of 2 oz every 3 hrs. So I waited until Noon to try and feed her again. Well, she vomited again. So I gave her about 30 min and attempted to feed her the rest of the bottle. It was at 12:30 that she vomited a 3rd time. I was starting to worry. I, of course, knew I had to prick her little heel and see what her Blood Sugar was. When I checked, it had reached an all time low of 62. Having been educated on LCHAD, I knew that if it reached 60 we were to go directly to the hospital and on the way give her some oral glucose gel. This is to temporarily raise her sugar levels. We are supposed to give 15 grams. Well, I tried and it went all over the place. This sticky substance went all over her face and all over me too. Come to find out, the 15 grams is for a child not an infant and the little that she got raised it up to 93, Praise God.

We got to the ER and we got registered. I gave them the protocol letter from Dr Marble, her specialist in New Orleans. Of course they read through it, then gave it back to me. This letter states that # 1, when her sugar gets that low, we are to go to the ER. #2, when we get there, they are supposed to IMMEDIATELY administer an IV of D10, basically a really concentrated dose of glucose.

Now at this point, she had not eaten, effectively, since 3:00 that morning. With any other child this would probably be ok, but not for an LCHAD baby. She was basically starving and had no fat to sustain her. So, it took them 2 hours to give us something. At this point they actually decided it was best for her to receive pedialyte and send her home! I was outraged. The letter specifically gave orders that they were obviously not following. I gave her the pedialyte, so she wuld get SOMETHING but at the same time was on the phone with the specialist. Luckily, we had his cell. He was pretty put out because the ER had not bothered to call him about her. He had to call up there and have a heart to heart with them. I was very grateful. She was still crying and I could tell she was hungry but there was nothing I could do. To shorten the story up, it took them about 4 hrs to get the IV in her, which we should have received immediately. We stayed there until about 7:00 at night meanwhile, earlier, we had begun to give her 10 ccs every 30 min. This obviously was not satisfying her. She was so tired and very hungry. It was so hard to sit there and try to console her for hours, knowing that she was so miserable.

Her pediatrician, Dr Vaughn, decided to admit her to the hospital to try to figure out why she was vomiting. They ran all sorts of tests on her, including some for her heart, to rule out myopathies (heart muscle disease) See with LCHAD, undigested fats can build up around the heart and eyes too. We were relieved to find that he heart looked normal. These tests will have to be repeated routinely.

We thought we were going to get to go home on Sunday, but Dr Vaughn ordered one more test to see if she had reflux, and Praise God she did. We had a small problem but it is easily fixable. She now on Reglan 3x a day. We were able to go home at about 3:00 Monday Afternoon.

She had a slight relapse yesterday and vomited 2x after the 9:00 am feeding. I was nervous because we just got out of the hospital Monday and I definitely did not want to go back. So I called up Dr Vaughn and he told us to modify the feedings again. This helped tremendously, even though it is exhausting feeding every 30 min. But, anything to keep Evie from going back.

One of the hardest things about this is knowing that at any point, we could end up back there. It could be for vomiting again, or an ear infection. It could be literally anything. The odd thing about her condition, is that she looks perfect. Aside from being small, she is fine!

I would not trade Evie for any other child. She is beautiful and definently a gift from God. I heard another mother say that she was grateful that God chose her and trusted her to watch after such a special baby. I am having problems dealing with this. And, forgive me for saying this, but I have asked, "Why us?" I am just determined to love her to the best of my ability and give her the best life possible. We may not be able to buy the best things for her, but we will love her unconditionally.