NG Tube Free

Sorry for the long pause in updates. I was very much inside my own head right before Charlie's Gtube surgery. I was so nervous but she handled everything beautifully. While we are still healing and trying to get past the pain and the adjustment to Gtube feeding, things are going relatively well. Its nice not having the stress of the NG tube but Im not quite there on being comfortable about the gtube....she is still hurting, wretching, etc. I feel like once we get past this, things will be better.

Once I find out what anesthesia they used I will post it here. They said I needed to go through medical records to request that information. She used torodol and tylenol for pain. We are still on tylenol for pain but i feel like she can start weening off here in the next few days.

The Countdown

The final countdown to Charlie's surgery has begun. 6 more days. I'm so nervous, excited, etc. It couldn't come soon enough. Now its to the point that even with mittens on, Charlie is still managing to pull the NG tube out partially. So now if I am not watching her, I have to swaddle her arms down so she can't get near her face. It makes me feel so bad because she HATES to be swaddled. Although after a few minutes of being really upset, she seems to calm down.

I also have been tinkering into essential oils to help calm her down. I cut some lavender oil with jojoba and have been putting it on my hands when I do anything with her and also massage her feet with it. I can tell a big difference. She slept way better last night (not as much tossing/turning) and when her teeth are bugging her, her tantrum episodes are way shorter (from an hour and a half to maybe fifteen minutes).

Her PT went fantastic yesterday. The physical therapist said she has fit a month of development into a week and is blowing her away at how fast she is progressing. Now she can roll super easily from back to front and has some awesome strength building in her neck. She tells me we may see some stalling once she has her surgery because she will be hurting for awhile but I hope she keeps marching forward like she always does.

We have a meeting with the feeding specialist during her OT today. I am anxious to see what they say about what they think is going on with her feeding. I'm lucky to get her to take 5mls and once a day we typically get a better feed of about 20-30mls. This is such a setback from where we were (she was taking about 2 ounces before) so obviously something is going on. They want another swallow study. I really hope she isn't aspirating. One more thing to worry about.

Thats pretty much it for the update. Hopefully we can get some answers soon. I cant wait to take her NG out for the last time....get that tape off her face and be able to see her entire face...been way too long.

Finally, Non-Irritating Tape Remover. I Must Share the Good News.

When we were admitted at the hospital last time, Charlie had a pretty bad reaction to the adhesive remover they used. She broke out in rashes. We have tried a few different kinds. The wound care specialist came to look at her blisters and said "You have to use this" and brought us a can of "SensiCare": SENSI-CARE Sting Free Adhesive Releaser Spray, 50ML. It seriously is amazing. You spray it on and the medical tape comes right off. I have actually changed the tape on her face without her waking up with this stuff.

I thought I would share this because I know a lot of our kiddos have to have medical tape on a lot and its not only irritating to the skin but I know it scares our kids. This has been a lifesaver for us.

Added Links To Supplements Page

Hey all! Charlie is doing great. Countdown 2 weeks to Gtube surgery. Also I have been starting to take her supplements in a little experiment to see how I feel. So far I feel pretty great, I have been taking ubiquinol for a little over 2 weeks and the other supplements for a few days. I have added links to the supplements page on which ones I am currently using if you are curious. There is debate on whether carriers can have mild symptoms. Since most of the side effects of taking the supplements are pretty mild, I thought I would give it a shot.

Here is a great video of Charlie playing with my cellphone:

Busy Busy Busy

Its been a crazy few days. Charlie is back into her physical therapy schedule and we have been trying to just adjust now that we are back from the hospital. Hospital stays seem to throw everything off kilter for awhile. The housework piles up and it takes about a week to get caught up.

Charlie is doing absolutely fantastic. Her physical therapist is so pleased with her progress. She is now grabbing things, trying to roll over (shes SO close) and is tolerating (maybe even enjoying?) tummy time now. We still have the issue where she throws up every. single. morning. It breaks my heart because she doesn't even cry much anymore. She just throws up and groans. We are experimenting with some nausea/vomiting meds but they don't seem to help. I really hope the G-tube and continuous night time feeds help. We have opted not to get a fundo (its surgery on the stomach that prevents throwing up) I have hope that we can get this under control with reflux meds or at least she will grow out of it when we start solids.

Life has been better. My amazing husband gives me "Mom's day off" every Saturday where I can go

out and do whatever I want. I usually do something active and then go grocery shopping. Today I may go hang out with my amazing friend Irina. She is just such a warm, caring and selfless person. Here she is with her amazing adorable puppy "Bear". She has definitely helped me so much with what has been going on. I also have gotten back in the gym which helps my mental well-being. I'm excited to get "me" back. I feel like I can be a much better Mom when I'm more comfortable in my own shoes....and pants.

For once I feel slightly better about the future. Maybe this is doable. It's going to be hard, but I always have been the type of person to choose things that were difficult anyway.

Hospital Stay

Sorry it has been awhile since the last update. Charlie was in the hospital for a few days due to a stomach bug. She threw up 5-6 times in the morning and just was not acting like herself so off to the ER we went. This time was so much more smooth than the first time. I felt like I knew what I was doing, the doctors and nurses knew us more and we were able to get D10 without a big fight.

Charlie was already a bit acidonic just from throwing up a bit and dehydrated. I'm glad we went and it was just 3 days which is the shortest hospital stay yet. Lets hope that she is good now and we can just remain home until her surgery in July for the gtube! I learned so much from our first hospital stay, the first time really is the hardest. We also got a lot of the same nurses as we had last time which helped not having to explain everything over again. The nurses we got were amazing too. Especially  two named Ashleigh and Tinsley who really made life a lot easier while we were there.

Nurses can make or break the stay and this was the first hospital trip where I had to leave for a bit to run home and take care of the dogs. I was a nervous wreck leaving her but each time I came back and she was sleeping comfortably. Tinsley even tucked her in and turned on a channel on the TV that played lullaby music. Very sweet when people do little things that make a big difference.

Lets hope she is doing better. She just threw up again this morning, but a morning puke session is not out of the ordinary for us, lets just hope that it stops there.

Sick Baby

Sorry it has been a bit in between posts. I was dealing with a sick husband and then sick baby. She caught my husband's sinus infection and it has turned into a bit of a stomach issue as of this morning. We are just waiting to see if it turns into anything worse. She threw up a few times this morning but so far has held down her 2nd bottle. Gotta keep an eye out. Its always a worry, making sure she is breathing right, acting right, etc.

We are also waiting for her PT who should be getting here within the hour. We get therapy 3x a week which is amazing. I hope this early intervention really helps her catch up physically in her development. Shes gotten stronger and stronger and we are so excited to keep working with her so she gets even better.

With her being sick and fussy, I have been a bit on edge. I suppose the looming G-tube surgery and all the stress that goes along with it has me more anxious than usual. I have become really close with another mom who has a daughter with VLCADD and she just had Gtube surgery and everything went very well so that makes me feel better. I just can't wait till its all over with and we can breathe a little better (and sleep better too).

Keeping fingers crossed that she does not throw up anymore today and we do not have to call metabolic and go into the Dr.

Parenting on "Expert"

My husband always jokes with me when things get difficult that "We started parenting on "expert" mode." We are big gamers, so we crack nerd jokes on a regular basis. I definitely agree with this statement. This goes for all parents of FODs, parents with kids with special needs. We are warrior parents, advocating, learning, constantly worrying, teaching others. The list goes on. Its been a rough week in our community. One of our members lost their little girl. My heart breaks for them. This has been the second death in the month and I think about their families a lot. I know it isn't much but I hope it brings the families a little comfort knowing that their child is being thought about often. Most parents can't fathom thinking about losing their child, but it is something FOD parents are thinking about on a regular basis. If we didn't, we would lose our children. We have to constantly be aware that their lives are incredibly fragile. It takes a toll on our mental health. A lot of times I look at my daughter and burst into tears thinking that I may not have her long. It can all go awry, terrible, tragic in a short few hours. However, this also makes us better parents. We are involved, constantly there to support our children. Appreciating every single moment and really being present. We arent the parent sitting on the bench at the park on the iphone. We are the parents watching our child in awe, thanking God that we have made it this far.

I love the community that I have found. I have made friends that have made this journey just a little bit easier. Someone to lean on who really 'gets it' and understands. A few of my friends with FOD kids are going through a really tough time this past week. I ask that you keep them in your thoughts.

Isolation

I never really understood how isolating having a child with special needs is. I mean, having a baby is isolating in and of itself. You cant go out much because its a big affair, the weather dictates (too hot/too cold), but with Charlie I am paranoid about her getting sick. I think most of my friends are paranoid about getting her sick so they don't come around much. Which is appreciated but it still makes me sad. I was  am a social person, so not talking to anyone or seeing anyone all day is really hard. I think a lot about what I had planned and how different this life I am living is. I did not want to be a stay at home mom. No offense to those who choose to, but I do not like being at home all day. I worked so hard to get through law school, pass the bar, internships, countless hours perfecting my writing skills, it would make sense that I would want to make use of all that. I miss adults, conversations, critical thinking, writing, helping others, etc. Charlie brightens my day and helps me get through it but this is not how I imagined things going.

Charlie's metabolic appointment went well. Her oxaloacetate is apparently helping her and a lot of her organic acids went down. Problem is her hemoglobin went down too. Maybe...I am suspicious of that test. She is acting great, lots of energy, breathing fine...etc. I don't think that would be the case with a hemoglobin of 8.5. We have a date for the Gtube surgery at the end of July, I'm just hoping that it all goes smoothly. Hopefully we get her anemia figured out by then. One thing after another, it never stops.

Suffering and Joy

When you are faced with something so big, so unexpectedly difficult, you never know how you will react until it happens. I find myself mourning something I thought I would have, digging in and pushing through, angry that life can be so terrible, amazed that life can be so beautiful, overwhelmed by the intensity of love that I have for my daughter. Life is just a giant paradox of emotions, feelings, experiences.

I think a lot about a video I saw about a couple who lost their newborn baby at three days old. Watching their pain took me back to the day when we weren't sure if Charlie would pull through. The Father said something that totally resonated with me, and still plays over and over in my head. "Through suffering is the most intense joy." or something to that extent. I am not sure if most people really 'get' it, feel what he is saying. I remember sitting next to Charlie's bed, her laying there and watching my Husband stroke her forehead and although we were suffering more deeply than anyone could imagine, I was so incredibly joyous that I could see him be a Father, even just for a little bit. I always heard that there is nothing greater than a parent's love for their child. Its so cliche but its true. And those without children will be like "Yeah, I get it" but you don't until you are living it. I don't say it to be 'holier than thou' type of statement but its just true.

Anyway, I am thinking about this entire thing because its Father's Day and seeing my husband be a Father to our daughter has made me incredibly joyous, even throughout this entire period of intermittent suffering. There is nothing wrong with suffering, its life. Life is to suffer. Life is to be joyous. Its because of that suffering that makes the joy all the more intense. I am so proud of my Husband. He is an incredibly wonderful man who has been a pillar of strength to me and is as patient as a saint. Cheers to all the wonderful Dads out there.

Typical Day

Its been a pretty quiet week this week (I LOVE quiet weeks) and I have enjoyed getting into a good schedule with Charlie. We wake up, eat, play and then its time to wake up Dad. Then while Dad plays with her I get to clean up and do some chores, sometimes take the dogs for a long walk. Then off Dad goes to work and I take over Mommy duty. Afternoon nap, sometimes I get a visit from a friend and then bath time and bed! Phew. Wow, that sounds pretty normal! I love that!

We may have gotten a date for G-tube surgery for 10 days from now. I am sort of shocked, nervous, scared, excited, happy, etc. for it. I am very scared about her going under anesthetic. I am glad the surgery is only 30 minutes long. It will be the longest half hour of my life, thats for sure. Very excited about Father's day this weekend. Thats pretty much it as far as this week's events. I really savor these quiet weeks. Its nice to just be able to relax and enjoy being a mom for awhile without having to be 'medical mom' constantly.

Day at the Museum

This past weekend we went to the Natural History Museum. Its a place near and dear to both me and my Husband's hearts. I absolutely LOVED going to the Houston Natural History Museum as a kid, and Tommy practically grew up at the one here in San Diego. We decided that we had to take Charlie now that she is starting to notice things that are around her. I am always nervous bringing her around big crowds, but I feel like she is now strong enough to venture out a bit. We kept her in her stroller and away from other kids to minimize the risk. She had such a blast.

She had so much fun checking out all the animal exhibits!

She got a bit freaked out by the hanging skeletons.

Something that sort of irked me when we took a lunch break. We got some weird looks when setting up her food pump. Yes, its different but please don't stare and whisper. I don't want pity either, I just want to be a normal new Mom who is dealing with slightly different circumstances. I think every Mom wants to just feel normal, whatever the hell 'normal' means. I'm so incredibly lucky that all I have to deal with is a feeding tube. I know there are a lot of Moms out there dealing with IVs, oxygen, feeding tubes, etc. I try to repeat that to myself when I get frustrated with having to carry the feeding pump equipment. These blog entries may go off on tangents by the way, I'm going to use them as sort of a venting mechanism. I know my close friends are getting tired of being my vent release all the time, plus I don't always want to be negative around them.

Anyway, this week will be quieter which is nice. Only thing is a follow up appointment with the pediatrician for some anemia we had going on last week and a visit by our amazing hospice nurse. Yes, we are on concurrent care hospice. It sounds a lot scarier than it is. Essentially because she has a very serious disorder, we qualify for hospice care. Its quite an amazing thing because they help us get medical equipment and medication all delivered to the house. They also have helped me with medical benefits that Charlie qualifies for to help her throughout her life. We are able to get therapy three times a week for her! Our nurse is also the sweetest person ever and has helped keep us out of the hospital several times. If you ever want to donate to an amazing organization, Elizabeth Hospice is amazing. I encourage anyone with a child (especially baby) with GA2 to look into hospice care. It helps so that you dont always have to leave the house to get supplies or if your child is just a little sick, you may be able to treat at home. 

We have some friends visiting from out of town so hopefully I can meet up with them and get out of the house too. Anyway, Cheers and Happy Tuesday everyone!

Feeding and GA2

Charlie, in addition to her GA2 also has a very small cleft palate. Its not even noticeable unless you look in the very back on her throat and it looks like she has a split uvula. It actually looks sorta cool...anyway, that coupled with the feeding issues due to her metabolic disorder has made the bottle a nightmare.

When this whole mess started and I was still in the hospital I got a lot of not very understanding people who kept chanting to me "Breast is Best!" not even considering that only breast milk was NOT best for my child because of her disorder. I know that some children with fatty oxidation disorder still are able to breastfeed but each child's tolerance of fat is different and Charlie did not do well on just breastmilk. I pumped religiously to keep supply, and we found that she could tolerate some breastmilk! I was happy to give her the immune boosting benefits. Aside from nutrition, there came a point where she was taking almost an hour to eat 30-40mls (about 1oz) which is not efficient for her. She was losing weight due to all the energy she was taking up by eating by mouth. The doctors let me know that putting an NG tube in would be beneficial.

At first I did not want an NG tube. I felt like she could do it, this would somehow prevent her from ever eating by mouth again. I agreed to have one put in but stressed that I did want her to keep feeding by mouth and then whatever she didnt take within 30-40 minutes, we would put through her tube. After that she started growing! She was gaining weight, slowly but surely and seemed to have more energy throughout the day.

We have had the NG tube now for about 3 months. It has helped her grow like a champ. She has gone from the 15th percentile to 50th! I place it myself every 2 weeks but now that she is getting older, its getting harder and harder to keep it in. She is confined to wearing gloves most of the time to prevent her from pulling it out. Putting one back in is stressful and tortuous. I am paranoid about her pulling out her NG while the pump is going and aspirating. Anyway, the risk of the NG is outweighing the benefits now. We have decided to move to a G-Tube.

This was an incredibly hard choice because I really did not want to put her through surgery. Anesthesia is more risky for kids with metabolic issues. But the benefits of a G-tube for a child with an FOD is a long list. She sleeps through the night so I could set up a continuous feed and sleep instead of having to wake up every few hours to set up a feeding by NG and worry about her pulling it out. If she is sick, it provides an easy port for feeding and meds. I wouldnt have to put her through the painful task of replacing the tape on her face every few days, the scary process of replacing the NG tube every other week, she wouldnt be annoyed by something on her face, plus people wouldn't stare as much. I don't mind, people are curious creatures, but I know that it will be an issue when she is old enough to care.

I know a lot of parents worry about the tube, worry about their kid being 'different', and it is not an easy choice to make.  However, unfortunately I foresee feeding (at least for now) being a constant struggle, and I want to do what I think is best for her health and well being. And the G-tube for our family seems to be the best option. We are waiting for a surgery date right now. I would appreciate any input from families who have a g-tube, I really don't know what to expect.

The Purpose

That day keeps flashing back in my head. Still sore, tired from giving birth less than a day ago, worried and walking towards a bed and seeing my perfect little girl covered in tubes, lines, leads, not moving. I remember the sad faces of the nurses that kept asking me if I had "support". I was so confused, why were they asking that. Why do they keep asking my religious affiliation? We didn't know what was going on for five days. Those were the worst five days of my life. I didn't sleep, I barely ate, barely drank. I really don't remember much of it. Days bled into nights as I clung to the hope that everything would be ok in the end. Once we got our diagnosis for Charlie of GA2/MADD, I scoured the internet for every shred of information I could get. I felt almost if I knew everything there was to know, then maybe I could help her, maybe she had a better chance. However, everywhere I turned I got the same cold information:

"Unfortunately, some newborns that develop severe symptoms during the first days of life usually do not survive for more than a few weeks or months because they have the very severe form of the disorder, called Neonatal Glutaric Acidemia Type-II."

I cried over this statement more times than I could count. Until I found the fodsupport website and the facebook support group for GA2, I didn't know there were other kids diagnosed as newborns and are still alive and doing well. I have met an amazing group of people who are raising kids with GA2. It is not necessarily a death sentence. No doubt it is an incredibly terrible disorder with long lasting health effects (she will be very high risk for heart and liver issues among other things) but I have faith that gene therapy is the answer and we just have to hold out till it becomes a possibility for us. 

The purpose of this blog is I want to show the family side of GA2. I really connected with other FOD (fatty oxidation disorder) parents who blogged about their trials and tribulations in having a child with a similar disorder and I found comfort in reading stories about other parents with GA2 kids. So this is my small way of giving back. I will post helpful materials, information and stories about Charlie and our journey with GA2. Feel free to email me if you have any questions or just want to connect with a parent with GA2/MADD.