LipiStart

February 2009

When Sarah was 6-months-old, the dietitian wanted us to try weaning Sarah off the elixir and on to Lipistart. This change was not due to Sarah's age but because Lipistart was a newer product and would be easier to use. The dietitian said that other dietitians that collaborate over the Internet were saying that Lipistart was working really well.

Here are my messages to the dietitian because she wanted to know how things were going:


2-6-2009

"Well, I tried just straight up Lipistart and Sarah ate 3 ozs. and then stopped and started fussing. I tried again with the same results. Then I tried a third time (each time was a different feeding) and she only ate 1 oz. then I mixed it with elixir so it was half and half and she drank most of it. So I am now feeding it to her 1/2 and 1/2 and will slowly increase the Lipistart in the mixture over the next few days and see how she reacts. :~)"

2-11-09

"Today, Sarah "chugged" her first full Lipistart bottle. Yeah! :~) My mom said she seemed gassy afterwards but I am thinking that could be because we just switched her over to the fast flow nipples because it seemed like the Lipistart might not be flowing out of the nipples as easily as the elixir did.

"As to her usual volume question she was drinking the same volume on 1/2 Lipistart and 1/2 elixir. I have noticed that her "usual" is changing some because of the solid food. She is drinking slightly less but not enough of a difference that I got concerned. Her usual now is anywhere between 4-8 ounces at one bottle feeding time. I've found it tricky, at times, to schedule the bottle feedings and solid feedings so that she eats a good amount at each. She seems to really like solid food and on one specific occasion refused her bottle but ate solid food and then drank a small amount to wash it down. I figure we can discuss that more at our appointment on the 24th."


It took us about a week to switch Sarah over to all Lipistart. In regards to the bottle nipples, we switched her back to slow or medium flow. While the Lipistart is thicker in consistency than the elixir, Sarah seems much more gassy on the fast nipples.

Lipistart is much easier than the elixir because Lipistart is just one powder. It's like normal formula just with only medium-chain fats instead of long-chain fats. It contains MCT in powder form and this makes it so we can use plastic bottles, if we want to. We can make the amount of bottles we use in a day (3) and store them in the refrigerator for up to 24 hours. When it is time to feed Sarah we just take out a bottle and warm it up.

Starting Solid Foods

At 5-months-old we started Sarah on solid food. We started with the basic rice cereal. I just mixed it with breast milk or elixir and then later, Lipistart.

Sarah needs to be on a low-fat diet in case she has CPT II. This means no avocado, which is a recommended soft food for "normal" kids because it is good brain food. Other than that, I didn't find many limitations for my choices of solid foods to puree.

Foods Sarah has been introduced to:
rice cereal
oatmeal
peas
carrots
green beans
banana
mango
papaya
applesauce
pears
sweet potatoes
zucchini
sweet corn
peaches
kiwi (no seeds)
cantaloupe
watermelon (seedless)
extra lean turkey
extra lean ground beef
lentils
cauliflower
broccoli


Finger foods:
non-fat Kraft cheese slices
wheat bread pieces (sometimes toasted)
Gerber puffs
Gerber yogurt melts
Cheerios
Rice Krispie cereal
celery sticks for her to chew on
banana slices
mango pieces
pasta noodles
Gerber apple flavored Wagon Wheels

Um . . . Really?

I happened upon some data on the the National Newborn Screening Information System website ( http://www2.uthscsa.edu/nnsis/ )which I had already been made aware of by my wonderful father-in-law but just now realized what some of the information means.

In 2008, 3,338,284 newborns in the United States were screened for CPT II. Of those newborns there is only one confirmed case. The data that I just realized is the category "Newborns presumed with condition." There are 163 in this category and 48 of those are in California. http://www2.uthscsa.edu/nnsis/ReportTotalScreenResults.cfm?testgroup=F&testid=F2&grouplevel=D&typegroup=0

This means there are 162 other babies in the country in the same situation as Sarah. These families with newborns presumed to have the condition are treating there child as though they have a disorder to prevent anything bad from happening but at the same time fearing the day their child will go into crisis yet knowing that this will be the only way to prove their child has CPT II. (I realize that some doctors choose not to treat these kids depending on what their blood levels were so this last paragraph was me speculating - I at least know this is the situation we are in).

I looked at other disorders to see if they had similar information. Cystic Fibrosis 6,442 are presumed to have the condition but only 393 were confirmed. Wow.

This is not ok.

Is it Denial or is it Hope?

It seemed like ever since Sarah was born medical people have been treating Sarah like she isn't a "normal" baby. This may have been a blessing considering she was being fed every three hours in the NICU because of jaundice. This was before the Newborn Screening results were known. If she truly has CPT II this treatment and concern was to Sarah's benefit.


When I look at her, I realize there is a possibility that something is mutated on her DNA but it just doesn't feel like we truly have anything to worry about. I get frustrated that we are treating her based on fear of the possibility. Yet, if we don't treat her, something could go wrong and then we would feel horrible for not treating her while knowing there was a chance something bad could happen. If she doesn't have CPT II and we are treating her as though she does have it, then: she will not get a good night's sleep until we can give her corn syrup before bedtime; she will not be fed foods (like avocado) with "good fat" for brain development or just plain yummy foods like olives and cheese; and she is being given carnitine three times a day and this is not natural and seems like it would hinder her body from properly producing or recycling (whatever the biochemistry of carnitine is) carnitine in the future (disclaimer: the last statement is not backed by any scientific or medical evidence, it is just a possibility that I have wondered about). I don't like the possibility that we are hindering her development because we are living in fear.

I am very aware that I may be in denial. I don't think I am (haha, double denial). I am simply a mother that wants the best for my daughter. I want proof. Show me evidence that confirms or refutes whether Sarah has CPT II. A diagnosis of "Presumptive CPT II" is not acceptable in my book. This tells me there is still hope that she does not have CPT II. For Sarah's sake, I will treat her as though she has CPT II realizing that there are negative consequences and yet I refuse to ignore the hope and possibility that she doesn't have CPT II and is a "normal" baby.

Here is something I posted to the FOD support group on October 8, 2008:

"I am new to this whole FOD experience and this has been my experience with questions (this is me venting also :~) ). My baby is almost 3 months old. I find it awkward when people I meet ask about how well she sleeps or if I am breastfeeding or bottle feeding. I'm still trying to figure out the best way to respond. How much information should I disclose? It feels like when people ask "How are you?" and if things aren't going well they really don't want to know. I have found saying, "She is a good sleeper." is the easiest and it is true. For the feeding question we just say we are breastfeeding and bottle feeding. If people probe more then I try to explain, what I understand, about Sarah's FOD.

I get the "Will she grow out of this?" question a lot. Also, they comment that she looks so normal or healthy that they can't imagine anything could be wrong with her. I have had two people respond to her like she was mentally disabled or that she would act abnormal because of their misunderstanding of her genetic disorder. In both cases I tried to respond with grace and did not make a big deal out of their treatment. Although as Sarah gets older, if similar situations happen, I will need to correct their misconception.

I am trying to treat her like a "normal" baby. My doctor even said she is a "normal" baby. This gets confusing for me though because she really isn't "normal" because we must have her on a low fat diet, give her carnitine, and make sure she doesn't sleep for more than 5 hours. The doctor says that when she is ill or not eating is when we need to worry. So far, she has seemed like a normal baby to me. How will I know if something is going wrong with her muscles or heart? How much information do I need to disclose if I am leaving her (already well feed) for an hour and a half in the nursery at church?

When my husband and I got married many people commented that we would have beautiful babies because we had such great genes. I find it ironic now, considering how rare this disorder is, that it just so happens my husband and I must be carriers. She is a beautiful baby though. :~)

Lisa
mother of Sarah, presumptive CPT II"

I didn't post these pictures on the FOD site but I'd like to show off how cute my kid is. :~)

The last picture was taken by the fabulous photographer Becky Fisher - http://www.beckyfisherphotography.com/Home.html

Constipation Frustration

This was something I posted to the FOD site on September 9, 2008. It was my first post to the support group:

"Hello, My daughter is 2 months old and was flagged by the newborn screening as possibly having CPT2. We are feeding her a mixture (we call it "the elixir") of 50 grams Polycose, 10 grams ProViMin, 7 mL MCT oil, and water to make 11 ounces. One feeding we give her the elixir and then within 3-4 hours later I breastfeed, that is our pattern. Anyways, I think the elixir makes my daughter constipated. I'm thinking maybe it has to do with the type of iron in the ProViMin. On the elixir diet she can go 1-2 days without having a bowel movement. When I breastfeed alone she has a bowel movement basically every time she feeds or frequently throughout the day. Twice we have taken her off the elixir diet and just breastfeed for 2-3 days and after about a day she becomes regular again. When I have mentioned this to doctors and dietitians they either seem to ignore my concern or claim the elixir can't do that and recommend I ask my pediatrician. Considering I can't feed her just breast milk for fear of what too many long-chain fatty acids might do to her, we just feel like we have no options. I will follow the dietitians advice and ask my pediatrician at her appointment on Monday.

Anyone notice a similar problem or have have any thoughts about my hypothesis?"


When I asked her pediatrician, he said that a normal baby can have 1 bowel movement after every breastfeeding or they could go up to 7 days without a bowel movement (seems like too long in between for my comfort level). He said what matters is the consistency of the poop. If it is really dry and hard then that means Sarah is constipated. I stopped worrying then because the consistency was good. About a month or two after this post on the support group, Sarah seemed to have regular bowel movements.

*I wonder how Sarah will react when she is older if we ever tell her that I discussed her bowel movements on the Internet. :~)

This is a picture of the look Sarah would make when she was pooping. We called it "Blue Steel."

FOD Family Support Group

September 2008

My father-in-law had mentioned that there was an online support group for people or family members of people with fatty oxidation disorders (FOD). CPT II is considered a fatty oxidation disorder. In September after the doctor labeled Sarah as having CPT II, I joined the support group.

http://groups.google.com/group/FODsupport

I also joined a group specific for CPT II.
http://groups.google.com/group/CPT2?lnk=

It is a great way to discuss issues involving treatment and diet and to learn about what other parents are dealing with.