If My Child is Sick – Fatty Oxidation Disorders Family Support Group

Many families often learn about FODs the hard way ~ when a medical crisis occurs. In the midst of chaos, parents’ stress levels are stretched beyond the limit! Not having an individualized written treatment protocol for doctors to utilize in a medical crisis can have huge implications, but so can how a family addresses the everyday needs of an FOD child/adult.

We hope this section will provide some helpful and practical suggestions for dealing with daily health issues, as well as with intermittent medical situations, to ease some of that anxiety.

Most of these suggestions/questions are offered by families in our Group who have been dealing with an FOD for years and have, over time, come to learn what works and what doesn’t work for their child. Please be aware that these are only suggestions and may not be appropriate for every FOD child/adult. Be sure to discuss your concerns or possible adjustments in treatment/diet with your medical professionals before making any changes.

Please contact us if you would like to see a new subject added to this section.

Subject: Blood Sugar Levels

Q. I have a question about low blood sugars and about not relying on them as an indicator of crisis. Hypoglycemia is not an indicator in all crisis situations. This is very important. I think this is a point that needs to be emphasized. Can someone elaborate on this?

The take home message from the Orlando Conference (Oct 2002) about low blood sugars was that for a long time, all FODs have been lumped together in a category of “hypoketotic hypoglycemia” – low ketones (or no ketones) and low blood sugar. Dr Roe, Dr Korson, Dr Rinaldo, Dr Winter, and the other speakers, all clarified that as we learn more about FODs, of which there are now 18 known, we are seeing different characteristics in each. Some, like MCAD and one type of VLCAD, are prone to low blood sugars…some are not. Also, in children who have had experiences with low blood sugar in the past, that may be their main red flag. And, infants and younger children tend to have more frequent low blood sugars than older children. HOWEVER, especially in children who are recognized and being treated, their symptoms may include lethargy, some other type of altered behavior etc., as they build up the toxic fatty acids during an acute illness or prolonged fast. You need to speak with your Drs to clarify what they feel is the most important concern for you and your child, based on their diagnosis and responses during illnesses.

Low Blood sugar is a very important symptom for some FODs. Normal blood sugar is 70-120, it might normally peak slightly higher after a meal, until Insulin is released and drives sugar into our cells to make energy. Infants are very prone to low blood sugar for several reasons, the biggest being they don’t have good stores of Glycogen, the body’s way of saving sugar for later use. Most Metabolic Specialists protect Infants with FODs from low blood sugar by having them eat every 4-6 hours. As we get older, develop more Glycogen and some other hormonal things change, we become less likely to experience low blood sugars…but in children with certain FODs it is still possible. So, in the older children, raw Cornstarch is used to provide a very slow release of sugar over a long period of time, 6-8 hours. This is why yesterday, I wrote that “under treatment” even those children with FODs that are associated with low blood sugars, usually do not experience low blood sugars. For sure, the only way to monitor for low blood sugar accurately, if that is something your Dr wants monitored, is to check it after 6 hours of fasting. If you check a blood sugar while Cornstarch is still being absorbed or after you have given your child something to eat or drink – the blood sugar will appear normal even if it wasn’t and then your Metabolic Specialist has no way of knowing how best to treat your child.

Lynne A. Wolfe, MS, PNP, BC
lynne.wolfe@nih.gov
Metabolic NP

Q. What is the normal blood sugar range for a child? Does it differ from an adult? My daughter’s was around 50 the last hospital trip. Our doctor also recommended to give corn syrup (in a crisis) to bring her blood sugar level up on the way to the hospital.

Q. What blood sugar level is considered to warrant a trip to the hospital? We do not test our son’s sugar. Should we?

My daughter has MCAD and has had problems with blood sugar during episodes. We only check her blood sugar if she has not been able to keep food down. The doctor told us to call if the blood sugar gets below 60. We do not go by blood sugar alone though. We watch for lethargy and vomiting. She has had an episode where we recorded her blood sugar at 39 and the doctor recorded it at 79. We thought we had made a mistake since we don’t check her blood sugar very often. He told us that “it could have been her body’s attempt to dump all remaining sugar into her system.” If we had ignored other signs and checked the blood sugar a little later, the “right number” could have delayed a necessary trip to the hospital. We do not check her blood sugar on a regular basis. We obtained our “glucometer” during a hospital visit. Insurance covered the cost.

Melanie
MCAD mom

We tend to rely on other factors such as time elapsed since last eating and behaviour for our son who has LCHAD. Irritability would be a sign that it was time to eat although this would apply I think to most children. We were also told that BS was not a reliable indicator on its own. It appears that there is no substitute for a parent’s experience and intuition about their child. This is why so many of us end up frustrated following emergency admissions when we know our child needs a tube or a line NOW (and not in 2 hours when they have been seen by a series of professionals).

Jon
LCHAD Dad

My son’s blood sugar runs between 50 and 80 daily. It’s an abnormal day when his sugars are above 80. Today his high was 67 and the doctors don’t seem too concerned. We keep it up with cornstarch during the night since it’s a complex carb. We pay more attention to his behavior with the count than just the count alone. This doesn’t make me happy, but that’s what we’ve been told to do. Our other son has low carnitine and low blood sugar. We have been working through the diagnosis stage since Feb. We still don’t know what is really wrong with him. They say he has some type of mitochondrial/metabolic disease, but that’s about all they say. I want to add that corn starch may help…3 Tbsp. for a 27 pound child will maintain blood sugar for about 4 hours.

Debbie
Undiagnosed FOD mom

I don’t test our daughter’s blood sugar. In the past, she has had episodes where she was sick from her fatty acid levels being elevated without having a really low blood sugar, so I do not count on that to be an indication that she is getting into trouble. I watch for other signs of her progressing into “dangerous territory,” which for her include muscle pain. She also gets a strange odor, which I’ve discussed here in the past, and which her doctor believes is her body making “abnormal” ketones. I think every case is different.

Dawn
VLCAD mom

My son has MCAD and we were specifically told “metabolic crisis can occur with or without hypoglycemia present.” For us, we don’t check blood sugar. We’re told to watch the clock and get him in. When he is sick, I keep a log of what he eats, what time he ate it, and if I have a question about whether or not to bring him in to the hospital, I call the endocrinologist on call (24 hours/day) and ask his/her opinion. Then again, we live within a half hour of our choice of hospitals.

Wendy
MCAD mom

I wanted to let everyone who doesn’t know about it that there is something called “Insta-Glucose” that I order from our local Wal-mart pharmacy. My daughter’s endo doctor gave it to me for when she is not keeping anything down or doesn’t want to eat. It really helps because it is a gel and is absorbed through the tongue and cheeks so they don’t have to swallow it! This is really useful for her because her sugar can drop 20 points or more in 30 minutes and we have to drive 30 minutes to the hospital. Also, there have been times that they haven’t been able to get an IV in so we give it to her. (It is very sticky though.)

Joy
VLCAD mom

Nicholas was diagnosed with SCAD at 3 weeks old. He was going to be discharged from the NICU today, and then he got sick for the first time. He was very lethargic (he is usually very alert), had diarrhea and excessive urination. They started a glucose IV immediately, and he is doing better now. When they tested his blood sugar before they started the IV, it was at 326!!! The doctors say that they don’t know why that is, considering that SCAD kids are supposed to be hypoglycemic. Has anyone had a similar experience?

Jackie and Mike
SCAD parents

I’m not really sure on this one but I think I remember reading something about “dumping” ~ it’s the body’s last ditch effort to use the glucose available and it “dumps” it into the blood stream causing a misleadingly high reading. I don’t know that I’ve ever heard of one that high though.

Mary Beth
MCAD mom

You said that you know when Anna is not going to turn around and will need IV glucose. What are the indicators for you? Is it basically the lethargy or inability to get the blood sugar up? We have only had a handful of low blood sugars since Johnny was diagnosed and always kept him at home but needed continuous feeding (every 2 hours). At those times, his blood sugars had dropped to the 50’s and we had to do the continuous feeding for 10 days to 2 weeks to keep them up. Poor Johnny would drink and eat what we gave him but he would get so full and bloated. We would try glucose tablets but with all the medicine he’s tolerated taking over the years, he says “those are garbage, put them in the garbage.” Since diagnosis, he has only been hospitalized this past February with a low blood sugar and elevated ammonia. That time his blood sugar went from 52 to 46 to 36 with lots of juice and food trying to bring it up. We knew then he needed the glucose but I’m wondering if the other times we might have shortened the time needed for the continuous feeding. With Johnny, it has always been that once the blood sugar drops, it takes a long time to replenish. I’m not sure why. You’d think once the blood sugar got back up that it would stay up but not so with him.

Sue
MCAD mom

Anna does not have a feeding tube. When she gets sick or when she has vomited, we watch her closely. It is easier now that she is older and we can reason with her. If she presents with lethargy, she goes to the doctor or ER. We check the blood sugar, but we don’t use it as a sole indicator. If she has vomited and not eaten well (with no signs of lethargy), we start checking her blood sugar every hour. We also encourage her to drink water and eat Froot Loops (her sugary cereal of choice). She understands at this point that she has to have her finger stuck and she has to try to eat. If her sugars drop below seventy, we prepare to go to the doctor. If they reach 60 we go.

Anna’s episodes usually involve low blood sugar and eventually lethargy. Monitoring blood sugar is the “easy” part. Learning to read signs of lethargy has been a little harder. We have gone to the doctor and decided every thing was going to be all right. There are times, though, that we can tell that she needs to go to the hospital. Anna, as well as we, calls it going to get pumped up. We felt this was a fairly non-scary description for her, her siblings, and friends. Her hospital stays are usually just over night with IVs. Her first episode was before diagnosis. She was in the hospital for three days with just the glucose IV.

The IV carnitine makes a difference (in addition to the IV Glucose). “Reading” lethargy – this is hard to explain. Anna will become tired and slow to respond verbal commands. Her eyes will be “sleepy.” She eventually will not respond and her eyes will not focus on anything. Then she “sleeps.” Since her diagnosis, she has not had a blood sugar below 39, which still isn’t good, but it is better than 12. Diagnosis is the key. We know to watch our child now. That first stomach virus kept us from going to the doctor when we should have. We were probably 24 hours gone too long. My non-MCAD child had been sick and slept a lot. I thought Anna was doing the same. Little did we know she was lethargic and semi-conscious. I wish we could get all doctors to learn about these symptoms and disorders.

Melanie
MCAD mom

Johnny also gets lower blood sugars with exertion and especially heat and not usually with illness unless his food intake has been drastically decreased. He seems to make it through the night now okay with the cornstarch before bed. When he was younger, I kept a sippy cup (Tupperware used to have great ones) by his bed with juice. Even now, because he has to have his meds every 8 hours, he gets juice with his meds at 10:15 PM and 6:45 AM. I stretch the night dose an extra half hour just so we can get a little more sleep. Have to really watch those teeth doing that, though.

We just had an interesting thing happen with the blood sugars. His aide got blood sugars in the lower 70’s and so increased his food intake at school. That night, Johnny’s food intake increased by two to three times the normal amount with blood sugars staying in the 70’s and 80’s. This happened for the next two days and then everything returned to normal. While he was doing the mass consumption we were watching for symptoms of some illness but it wasn’t until the fourth day that he finally came down with a wicked cold. I know that they say it takes three to five days after contact to come down with a cold so I figured that somehow his body was telling him to get ready.

Sue
MCAD mom

Subject: Hospital Visits

Our pediatrician admits our daughter directly to the pediatric unit. I walk her through the front door, catch the elevator, and the nurses there are usually waiting for us, with orders the doctor called in. Our local hospital has an extremely small pediatric unit (usually no more than 6 or 8 kids)…that may be why they’re able to do this. In addition to the frustration of the ER, it also saves our insurance $$$$, and she isn’t exposed to as many sick people as she would be in the ER. Maybe you could check to see if this is possible at your hospital.

Gail
MCAD mom

We too have had difficulties with our local hospital. The last time we had to go to the ER we waited in the waiting room for over an hour. Luckily our son was taking sips of juice. Then I spoke to the attending physician about the wait and he said that he understood it was a problem but didn’t know what we could do. Our pediatrician even called ahead and the ER doctor was supposedly watching for us. Then after he had an IV (after 8 tries) for a few hours the ER doctor said we should go home. I informed him that our son had just vomited again approx. an hour before and I was not coming back again and letting them use him as a pin cushion again. That we would stay until he could keep something down. That is what we always do. He said he would have to clear it with our pediatrician. Which of course was no problem. We just stayed until noon or so the next day after he kept down cereal and juice. I get frustrated when we go to the ER and the triage nurses comment more on how cute he is than reading the protocol letter.

Lori
MCAD mom

I keep a bag packed at all times. In it I keep a t-shirt and shorts and underwear for me, two T-shirts for our son, two lightweight baby blankets (useful for throwing up in the ER waiting room and for naps), and basic toiletries (toothbrush, etc.). We also bring our own glucometer with us. That way I can check his blood sugar when they can’t find a glucometer or while waiting for hours in the ER. We have gone to the hospital administration (by calling the hospital operator) when we felt that he was not being treated appropriately. I find it helpful to leave the hospital for a couple of hours each day if he is stable.

Betsy
Undiagnosed FOD mom

Subject: Eating & Drinking Tips

If our son won’t eat, we take him out to someone else’s house. He frequently eats better somewhere else besides home. If he won’t eat or drink, we try to syringe feed him apple juice, when this gets to be too much, we hospitalize him for an IV. We are aggressive about getting him admitted before he starts dropping his blood sugar.

Betsy
Undiagnosed FOD mom

We don’t give pedialyte. We give white grape juice because it has more carbs. Something around 36 per 8 oz. If we keep up to one ounce in per hour than we can avoid the hospital.

Lori
MCAD mom

I keep a bag containing the B.R.A.T diet in our home at all times. A box of rice baby cereal, a box of Carolina rice, apple sauce and banana baby food. The first sign of diarrhea or vomiting, I start feeding him the B.R.A.T. including white bread toast and bananas. I’ll mix his Portagen Formula with Pedialyte instead of water. I’ll feed him tiny quantities throughout the day in order to give his tummy rests. If at any time his output exceeds his input, then I take him to the hospital for an IV glucose drip until he can hold down food again (usually 12 to 24 hours on IV gives his stomach enough of a rest and gets his electrolytes back to normal so he can start eating again). I also don’t like to wait too long before making the trip to the hospital because it is harder to get a line into him if he is slightly dehydrated. Be sure to find out how many calories your child needs to consume when ill to keep him safe.

Gina
VLCAD mom

When my child does not want to eat, I try to get her to eat a sugary cereal. Her cereal of choice is Froot Loops. Cereals are easy snacks to send to school.

Melanie
MCAD mom

Due to the fact that full strength juices can cause loose BMs, we always water down the juices. We do not use apple juice at all. For older babies: the baby brands of pear and white grape juices; For toddlers: 10% fruit drinks or watered down juices other than apple; For older children: “kool-aid” type drinks and small amounts (juice box size) of the 10% fruit drinks (like Hi-C).

Melanie
MCAD mom

Subject: Protocol Sheets

Keep your protocol sheets everywhere and anywhere (glove compartment, diaper bag, by the kitchen phone, caretakers and family members’ homes). My family practitioner had our son’s Protocol letter reduced on a copier to 4 inches by 4 inches and keeps it in his wallet at all times! I am sure it has come in handy the times we called him at 3 am! We also have a copy on file at the hospital. We also made a reduced size copy and taped it to our driver’s licenses in the event of a car accident, etc. Taking these measures greatly reduces anxiety during an emergency. It also gives us peace of mind and a feeling of control.

Gina
VLCAD mom

Subject: LCHAD & Eye Concerns

My daughter, age 4, has retinopathy in her eyes. It has not affected her vision at all. She gets her eyes checked every 6 months. She is taking cod liver oil, 1 ml, twice a day for her eyes. So far this seems to be working and her eyes are not getting worse. There has been no change in them since 16 months when it was first discovered.

Melissa
LCHAD mom

My son started to have eye problems at age four. Tomorrow is his birthday. He will be seven, which by the way is always a huge celebration for us, since he wasn’t supposed to live past one year or walk or anything, and he is now an active almost seven year old! His doctor had me take him to an eye doctor and they did a test on him that was done outpatient under anesthesia, and at that time he had diminished night vision. Then this last July, I took him in for his routine eye visit and found out that he could not see very well out of the front of his right eye. I was devastated. They had warned me when he was four that retinopathy is a disease that starts at a certain point in the eye and can eventually leave them almost blind, but I still wasn’t prepared for it. So I sent out an email to this list and they connected me with doctor Harding who is doing a study with DHA & LCHAD children with eye problems. He explained to me that because these children cannot break down fat that it deposits in the eyes because they are not producing the DHA needed to break down the fat. I hope that makes sense. We went to Portland, OR, in January where doctor Harding is and we will go back in July to see if the DHA is helping at all.

Julie
LCHAD mom

Regarding the RP problems for LCHADD children. It is my understanding that most kids with LCHADD will eventually end up with RP at some point in time. Apparently, DHA (docosahexaenoic acid) is a fatty acid naturally produced by our bodies when burning fat. Because LCHADD children have such a limited fat intake, they tend to have low levels of DHA. The retina is made up of various different fatty acids and DHA makes up about 40-50% of it. So, if a child with LCHADD is deficient in DHA they will more than likely end up with RP. I am not a doctor, but that is how it was explained to me.

There are two studies currently being conducted concerning DHA supplementation in patients with LCHADD. One is being done at the University of Wisconsin-Madison, overseen by Dr. Jon Wolff. That study began in Jan. 1998 and will come to a conclusion in 2003. A newer one being conducted at the Oregon Health Sciences University, overseen by Dr. Cary Harding, started in January of this year. I know that the one in WI is not taking any more patients, but I think they still have some openings in OR. You can contact Dr. Harding at 503-494-5799 for more information.

We took our two little ones in March of this year to OR. They did some blood tests, an eye exam, visual evoked potential (VEP) test, and an electroretinogram (ERG). The eye exam and VEP were simple and non-evasive. The ERG required that the child be sedated. This meant that the kids had to have an IV to prevent a drop in blood sugars. The ERG took about two hours from start to finish. Once the testing was done, we received a powdered form of DHA that we give them on a daily basis. They each take 1/4 tsp. once a day. I just mix it in their formula…they don’t know the difference. There is no smell or taste to it. There are no side effects

The study requires that the labs be redrawn again 3 months after the initial visit, a follow-up visit in 6 months, and then every 12 months after that for 5 years.

We were told that a majority of the children involved in the WI project showed signs of improvement. The remainder had no change at all. We figure that if they aren’t getting worse and there is a chance they will actually get better, it’s worth it.

The company that makes the DHA for the project is Martek Biosciences Corp. They do have DHA available to the public. It is the exact same thing used in the studies. We thought about just getting it from them, but we felt that if we could contribute to research, we should. You might ask your doctors about getting it that way. I am sure that Dr. Harding or Dr. Wolff would be able to give them a dosage. I know that some people give their children Cod Liver Oil. I believe the DHA from Martek is about 120 times more potent than the COD Liver Oil and it has no taste or smell or added fats.

Anyway, that is what I know about RP in children with LCHADD. Personally, I would contact Dr. Wolff, 608-263-5993 or Dr. Harding, 503-494-5799 for more information. They are the experts on this subject.

Kimberly
LCHADD mom

Subject: Breast Milk & Supplementation

Q. It’s the breastfeeding advocate again. Thank you for your response and my heart breaks for you and the loss of your baby. I’m just trying to explore this breastfeeding issue. There was something in one of the newsletters a while back about the importance of giving formula to newborns before the mother’s milk comes in. This disturbed me because I feel so strongly that nursing is the most important thing we can do for our children and if women start out with formula they are more likely to continue with formula.

Of course it’s not an answer to all problems and it didn’t save your child but nursing does prevent things like dehydration, allergies, intestinal problems etc, all things that are serious issues for FOD kids.

My daughter wasn’t given formula in the first few days of life and was okay. At age one she was, thank God, not so sick that she couldn’t nurse but a week of vomiting didn’t put her into a coma. When she wasn’t okay was at 2 1/2 when I had recently weaned her and she had her first vomiting incident. She wasn’t that interested in food and drinks and at that time we didn’t push it because it was pre-diagnosis, and she was in a coma the following morning. I don’t know, maybe it’s all a coincidence. Thoughts?

Vali
pending diagnosis:
Acylcarnitine Translocase Deficiency mom

Hey everyone! Just wanted to put my two cents in. I too am a huge nursing advocate. I nursed my first two boys until almost two and my third son until he got sick. But I had to supplement our third with formula because of poor latching, sucking, failure to thrive. It was a good thing I was not so hard headed as to not attempt supplementing. Also, I was in ICU for the first week he was home and not allowed to pump or try to nurse for a week after I got home. So the first two weeks of his life was strictly formula (a lot of it forced), and then we started in with the nursing. It took a little while, but then he got to where he was stronger and wanted to nurse more than take the bottle. I think if I had not forced him to take the formula around the clock, he wouldn’t be here today.

I definitely think that the La Leche League and all local stores, nurses, consultants, etc. should be “enlightened” to the FOD dilemma. It is one thing to be a strong advocate, it is another to be so stubborn as to possibly put a child’s life in danger. Before our Justin, you could not have told me that a bottle may be better. Now, I am open to all discussions. I think if we explain the problems and the consequences, people will be more apt to look for the possibility of something more serious than the lack of determination. I just think that with all of us being so few and far between we need to convey to as many as possible the fact that there really are medical reasons that may be at the root of a “poor nurser” or a “failure to thrive” and that if they are overlooked could potentially be fatal. As soon as the “packets” are done and I can get my hands on one, I plan on copying it and getting it to every nursing, pediatric, ER, OB, etc. entity in the Phoenix area. I encourage everyone to think about doing the same. The worst thing they’ll do is throw it away. The best thing they’ll do is share it with their clientele and spread the word.

Kimberly
LCHADD mom

I would like to put in my two cents worth (again) on nursing and supplementing. Like I have already said, I did not have any problems with my MCAD daughter when she was an infant. She was not diagnosed until she was 17 months old. When I think back though I wonder if things would have been different if I had not supplemented. She was a very good eater from the start. I could not pump so if she couldn’t be nursed for some reason, she would get a bottle. I only nursed her for three months or so.

I remember letting her go to bed with her bottle. Yes, that is a big No-No but in hindsight, it is probably what saved her. The doctor even said so. I do not recommend letting children lay down with a bottle, but I am glad I put mine to sleep with a bottle. One of my non-MCAD children had trouble nursing from the start (my oldest daughter). If I would have put her through what I put our other daughter through trying to get her to nurse, she would have had an episode at a much earlier age. I like nursing. I would recommend nursing. But I never had a problem with supplementing and neither did my kids. They would switch back and forth with no problem. I hope I don’t offend anyone. I have quite a few (La Leche type) friends. They were “appalled” at first. Each child is different (FOD or non-FOD). Let the mother do what she thinks is best for her child. This is just one of many difficult decisions that a new mom faces.

Melanie
MCAD mom

I would have to agree with you. My son was in the NICU for 89 days and I am sure some of you know what stress that is. I tried to pump and was able to keep up with his feedings until about a month before he came home. I tried nursing when he came home but frankly with all the other stresses of taking care of him, (feeding every three hours with formula, oxygen 24 hours a day, two monitors that false alarmed all through the night, and a mountain of meds and doctor’s appointments throughout the week) I just couldn’t keep up with double duty (formula and trying to pump). In addition I just couldn’t produce enough. I had a snotty nurse give me a guilt trip about it. Finally, I just had to stop. I had to maintain enough energy to keep my son and me alive! I find that the most energetic advocates of nursing are the ones who don’t really have a problem doing it. Don’t get me wrong. If I could’ve done it I would’ve. But in my case I just couldn’t physically.

Addison

My child (unspecified metabolic disorder) did not tolerate breast milk. I tried very hard to nurse and he would latch on and suck but did not get much. He did the same from the bottle when it was filled with breast milk or formula. When given pedialyte or apple juice, he sucked it down. Henry self-regulated his diet from birth. We now know that he doesn’t metabolize fats or proteins normally. Henry also will eat nothing when sick with a stomach virus and when not feeling well would have nothing to do with breastfeeding either. I do think that each child has his own food or drink that they tolerate better when sick. One of Henry’s favorites is rice cereal (even now at 28 months).

Betsy
Undiagnosed FOD mom

I can only speak from my experience but here’s my two cents worth. My son died with his first MCAD episode at three days. About four hours after his death my milk came in. My first son was breastfed. The big problem is that first-time parents do not know what is right or wrong when it comes to much of anything about newborns. Most of the time they are lucky and everything is fine. Ten years have passed and my memory of that time period is foggy, but I believe he was not nursing well from the beginning. Combine that with a circumcision and leaving the hospital when 24 hours old…a lethal combination for MANY FOD babies. I am not sure if the other FODs can present so drastically as MCAD did for us. Maybe breastfeeding without supplementing at the newborn stage is more of an issue with just MCAD babies. All I know is that I would give anything to know then what I know now!

Jacque
MCAD mom

It’s an issue with all the FODs–unfortunately those who think one should ONLY nurse NO MATTER WHAT–don’t know about these disorders and others like them (i.e. organic acidemias, urea cycle disorders etc.), and if they DID know about them and the consequences of an infant not getting sugar/carbohydrate calories from the very beginning (and not three days later when the milk usually comes in) they wouldn’t be so rigid in their thinking that ONLY nursing is the way to go. Professionals AND families need to be aware that supplementing (even if its just sugar water) may save THEIR child’s life someday!

Deb
MCAD mom

Hi, again. I’m one of those “La Leche types.” I sincerely thank everyone who responded to my questions. Your input really helped to shed some light on the subject. We’re all out their floundering to find answers to why and how this disorder impacts our children and looking for ways to prevent the loss of future lives. I will certainly contact some of my friends who are La Leche leaders and fill them in on what I’ve learned. I also want to point out that although some of those breastfeeding advocates that come on too strong are surely lacking tact, sensitivity and awareness of each individual situation, they are also passionate mothers out their fighting for a cause. I’m grateful that they were there for me when I needed assistance and support to nurse my children and I’m just as grateful that this support group exists while I swim in confusion trying to learn about my daughter’s disorder.

Vali
pending diagnosis:
Acylcarnitine Translocase Deficiency mom

I have bit my tongue for a long time over this issue, but now I must put my two cents in. I too wanted with all my heart to breastfeed. Read some of “The Womanly Art of Breastfeeding,” both my husband and I took a breastfeeding class at Kaiser and I mentally prepared myself for the ‘pain’ and the intimacy.

We came within minutes of losing our daughter when she was three days old; my milk came in the day she was rushed to Lucille Packard’s Children’s Hospital. But because we got a preliminary LCHAD diagnose back on her fourth day of life, the doctors were able to modify her IVs to reflect LCHAD, i.e. carnitine added and lipids restricted. As she was being weaned off the IV onto an NG tube and then a nipple she got about 25% MBM in her formula. We brought Ysabal (at 4.5 weeks old) home on Provimin, Polycose, MCT Oil and MBM. When she about five months old her essential fatty acids were too low so the MBM was limited to 50cc’s per day and canola and walnut oil were added. I ran out of pumped and frozen MBM just last week (at 8.5 months of age) so Ysabel is now on Portagen, Polycose, MCT oil, Canola Oil and Walnut Oil. She has been getting 11% long chain fat in her diet since February and shows good metabolic control. In fact she is now off the carnitine.

Our daughter is doing awesome and in fact ahead developmentally (this is the little girl they never expected to wake up and look around and showed brain damage on an MRI.). While I believe mother’s milk is best for most kids, I came to understand that too much for Ysabel was toxic to her body. How could I fill her body with something that may damage her? Using the MBM in the specialized formula was the best solution. No mother with a FOD baby should be shamed into breastfeeding and I would actually discourage it. Rather I would suggest that each family work with a nutritionist and add the MBM according to the child’s needs. Yes, I missed the intimacy of the feedings and sometimes it is hard for me to watch other mother’s breastfeed and yes, pumping milk was a BIG PIA, but it was what was best for Ysabel. And isn’t that what is important?

Lisa
LCHAD mom

Subject: Feeding Gastrostomies (G-tubes)

My son is autistic, has mild CP and wears leg braces in addition to having a g-tube. He is very uncomfortable being approached by other children with questions, even if I answer for him. Sometimes he just wants to go to the pool and relax and be left alone. No one else there is having to discuss their body parts. I know kids are curious, and we are matter of fact about it, but my son tires of the questions and it certainly doesn’t help the social problems of autism for him to be singled out as different by other kids, “special” or not.

Never mind that at pools that don’t know us someone always walks up and sees his bandaged g-button, assumes it’s an open wound, and starts off by saying “He can’t swim with that.” They don’t know what it is till we explain, and then it’s ok, but until then my son hears that he can’t swim. I have to do what’s best for him, not for the other kids, and right now that means minimizing the differences so he can swim in peace and not want to leave just because he’s being overwhelmed.

Kelly
VLCAD mom

My son had his mic-key g tube put in two years ago (at age four months). Nothing has to be done to it when he swims. It looks a lot like the part you blow into when inflating a beach ball. It took me two years to get up the nerve to change his tube, but now I do it and it is NOTHING! It is so simple! A little advice, carry an extra one with you at ALL times. If it comes out, you should get the new one in him ASAP (or else it is difficult for you and painful for him, the site actually starts closing!)

Gina
VLCAD mom

My son also has a mic-key. How often do you have to change it? His can go about 4-6 weeks and it starts getting clogged up. He has to be sedated and screams hysterically when the doctor changes it, his site also requires frequent cauterizing due to granulation tissue, and he really hates that. We used to do those things ourselves but it took two people to hold him down. We’d rather he sees the doctor as the bad guy, not us. Some nurses have told me to put coke through it but that didn’t seem to help. It could be his formula. It tends to clump easily. If you have any ideas for how to keep it from getting clogged, I’d appreciate it. Thanks.

Kelly
VLCAD mom

I bet the pain your son is experiencing when his tube is changed is due to the granulation tissue. Otherwise it should be fairly painless if all the water is taken out of the balloon. Our daughter also used to have this problem. One time years ago when her doctor changed the tube he actually went into the hole a teeny bit with the silver nitrate stick and she has not had any problems since! Also the fit must be near perfect…if it is too long and moves up and down a lot this is what causes the granulation tissue to rise. I am so sorry it is such a big deal for him…one more thing they must tolerate…are you flushing with warm (almost hot) water frequently? Our daughter’s last about four months. Hope this helps.

Lisa
VLCAD mom

My daughter has had her G-Tube for four years She started with a mic-key button but we had problems with the tubes becoming clogged and breaking so decided to try a MIC-G which dosn’t have this problem, providing you flush her tube occasionaly and change her balloon water weekly. It lasts for up to six months. She also finds this tube more comfortable against her tummy. Our problem starts when her tube needs changing. She has a very good stoma site and has never had an infection. She has the occational granulation problem but this is soon cleared up with treatment (I use sofradex eye or ear ointment).When we change her tube she needs to be held down and because she is so tense it makes it harder to pass the tube. It has become worse since last year when her balloon popped during the night. Her tube came out and by the morning her stoma had almost closed and i couldnt pass a tube at all we ended up in hospital for the day to have it reopened, but because of her chest problems they couldnt sedate her and it took three people to hold her down and several attempts to reopen the site. Since then it seems more difficult to pass a tube and she frets about her balloon popping all the time asking me to check it three or four times a day. She also hates having her stoma site cleaned and has to be held down and comforted. Regarding tubes getting clogged: I have also heard of Coke being used and you can also use a small amount of lemon juice plus regular flushes are a good way of stopping clogging and a good way of getting extra fluids into your child. I hope this is of some help.

Clare
GA2 mum

I talked with a Zevex rep here in Austin and they have a button that might solve this problem – it is made with a different material (silicone?) instead of rubber? Don’t know much but we are going to try it and I will let everyone know the results! The Zevex pump is the greatest thing since sliced bread! We do use the backpack also at night though – I fill the bag and turn it upside down to prime it – this gets all the air out of the bag – then put it on our daughter’s bed. That way if she needs me (she is upstairs, I am down) she can get out of bed and carry the pack. Plus I am hoping one day she will learn to wake up to go to the bathroom in the middle of the night – is anyone else getting frustrated with their “big” kids having to use pull-ups still? With all this liquid I sometimes feel like their bladders will never be trained! P.S. That is what is so great about the Zevex pump and backpack – The video that comes with it shows kids rollerblading, etc. and hanging upside-down on the jungle gym!

Lisa
VLCAD mom

We do the same. We just attach the pump (very easy to do) to the IV pole at night. While visiting last week, we just stood the backpack up in bed (propped it against the bed rail). It worked great. I think it is impossible to say if having a gtube would have prevented a hospital stay. You might have been able to put him on a slow drip at the first sign of stomach troubles, and this might of saved you the hospital visit. Having the calories he needed, given to him VERY slowly (to give the tummy a chance to rest, as opposed to a bolus feed from a bottle) might of done the trick, but if his diarrhea was severe enough, he would of need a total rest from food and just IV glucose to get his electrolytes back in order. My doctor wants our son to continue having his Portagen formula throughout a hospital glucose IV–stomach virus ordeal because she said that it is vital he gets the MCT oil that is on the Portagen (he is VLCAD not MCAD). A few times he was so sick that while at the hospital I took him off the Portagen for a SHORT period to give his gut a rest. The using his g-tube I would ever so slowly start to drip it into him. That is why I am thankful for the g-tube, because I am in control during a crisis. I am not saying a g-tube is the right decision for every family/child. But for him, it was a good choice, especially because he had MANY, MANY stomach viruses during his first two years. I did notice from reading these e-mails that most MCAD children do not have g-tubes, while it seems that many VLCAD and LCHAD kids do. That was interesting to me.

Gina
VLCAD mom

At the first sign of illness I hook our daughter up to a continuous drip of her formula. This has kept her out of the hospital on numerous occasions–if the gut can handle it I have been told that this is better than IV. Of course if vomiting or diarrhea is not controllable, dehydration will come quickly, then IV for sure. Each illness may be different.

Lisa
VLCAD mom

Our son has a mickey button. He started with this at 1/5 yr old and all it kept doing was popping (3 days/1.5 months). So the Doctor said we would try a Bard and we tried two types. We found them not very good as he moves a lot in his sleep and kept coming unconnected and he was not getting all his feed during the night. So we fought to get the mickey back. We have it now and it still pops (5days/ 2 months). Can anyone help with a different make of button?

Belinda
LCHAD mom

We have the Bard button and we had the problem of it coming disconnected also. We started putting split gauze beneath the button and then taping the tube to the button and gauze. It has not come out since. It’s been two years.

Kimberly
LCHAD mom

How much does your son take at night and could you give us your feeding times. Our son is doing 800cc over night and we have not been able to find a company that has the little pump/backpack with a feeding bag that would hold a large feed at night. We may need to talk about changing his times around along with amounts.

Teresa
LCHAD mom

I just wrote about my strong feelings for g-tubes, but I forgot to mention that our son eats a normal (low fat) diet, by mouth. However, the amount of formula he would need to drink is A LOT (400 cc every 4 hours), and I don’t know how you can get a 2 1/2 year old to drink when he isn’t thirsty! Besides, it tastes nasty (oils and carnitor and vitamins mixed in a water and powder solution…yeck! Never mind how it could damage his teeth, especially at night). For us, the g-tube and the backpack-feeding pump have been a true blessing. We love them and he is doing great. His CPK’s are perfect too.

His doctors (Dr. Roe from TX and Dr. Seashore from Yale…both specialists in FODs) agree that continual nighttime feeds, are not only helpful now (for various reasons) but they are also helping their chances for stronger muscles (less breakdown) in the future. It was explained to me that many times a child will deplete their glucose store (VERY easy to do…happens very quickly) and then we feed them to restore it…so their bodies are always playing “catch up.” With frequent feeds (He now wears an adorable little backpack hooked to his g-tube that feeds him 400 cc every 4 hours — it takes 1 hour and 15 minutes to run it into him) their bodies don’t have to play this game that is damaging.

The backpack looks just like book bag backpacks – no one knows what is going on, and I can relax and not have to worry about getting enough food into him…especially on hot days when all he wants to do is play outside and not eat or drink much. I just throw his backpack on and he goes about his life. He can do ANYTHING in it (except swim!). We tried different backpack feeding pumps and the ZEVEX is the absolute best – there is no comparison. It is so simple to use, that my 9-year-old hooks him (2 1/2 VLCAD) up all by herself – sets the pump’s computer and everything. I am just so excited about this pump, and would not of known about it if it was not for other FOD support group members…so I just wanted to pass it along too!

Gina
VLCAD mom

Our son eats normally during the day and the overnight feeding is to prevent periods of fasting rather than to provide supplemental feeding. I realize that he is quite unusual in still using an NG tube but as it works so well for us we have stuck with it. We did consider a G tube when he was a baby but it wasn’t warranted at the time (because he was so well) and we haven’t really felt the need since. Just as a matter of interest, of the UK families who use G-tubes (about half) many have had problems with them – often from the time they were placed – there seems to be quite a variation in the expertise available in the UK with some centres doing better than others.

Pam
LCHAD mom

We had our son (LCHAD) on an NG tube for about six months, when he was about one year old. It is doable and becomes easier with practice. Don’t be intimidated by the procedure, you will become very comfortable with it. Please be encouraged that the NG tube is not painful after it’s inserted. He has since then had a G-Button. The G-button is very easy to take care of also, even with swimming and other activities. It has been a hassle at times, obviously, but we know he is doing so much better because of that constant nighttime feeding. Otherwise, he gets achy and lethargic. We only used the cornstarch when we were in the process of moving. His formula at night includes: one cup of polycose powder, two T. MCT oil and two cups of skim milk. He will be starting 1st grade this year, so we will make sure a mixture of cornstarch and fructose is added before he takes off for school all day (along with making his own lunch). We believe that the continual infusion at night has enabled him to have a sustainable, normal level of activity all day long. Hang in there, you will have your ups and downs, but you’ll make it.

Scott
LCHAD dad

NG Tubes/Buttons

Ethan has a Microvasive button. Instead of a balloon, it has a hard plastic piece behind it so it absolutely cannot come out! The outside looks like the part of the beach ball you blow up. It means we have to have it changed every year but as much as Ethan plays with it and pulls on it, we would be going crazy with the other kind. You might ask your doctor about this if it’s something you think might be right for Rachel. Ethan is 17 months old so he plays with his a lot, but we’ve had it since March and have had no problems.

Mary Beth
MCAD mom

Jamie is 9 yrs old and G-Tube fed at 85mls per hour for a total of 20 hrs. This means she feeds right through the night via a flexi-flow pump that needs topping up every 2hrs 20 minutes with a 50 ml water flush every 4 hrs. Lewis (2½ yrs) has 4 X 250 mls in 24hrs. These are gravity flow feeds and take about 1hr 20 minutes ~ any faster for him and he vomits. With a 20 ml water flush before and after the whole process, it takes around 2 hrs. Emma the closest in age to your Nicolas, but still a lot older, is like Lewis ~ gravity fed 4x200mls per 24hrs. Emma was also tube fed at around Nic’s age and each feed with water flush would take around 20 minutes. But if night feeding is a real problem for you, but no night feeds represent a problem for your child, then my advice to you would be to ask your Dr for info about feeding pumps, work out Nic’s needs with your dietitian, set the pump at a suitable flow rate, switch on and away it goes ~ your baby feeds while you get some much needed rest with one ear ever open for the odd problem that can happen when feeding any young baby. Hope this may be of some help.

Woody
UK grandfather of GA2 x 3

Just thought I’d put my two cents worth in on the night feeding issue. Ethan has been on a pump for his night feed since his diagnosis at 7 months. He gets 40cc’s an hour for 10 hours. It’s wonderful. Once we figured out how to tape the connecting tube into his button so it wouldn’t pop out, we’ve had (virtually) worry free nights. Is there any reason why Nicholas couldn’t be fed with a pump? It would definitely be worth asking your doctor. I don’t know if feeding him 60cc’s in five minutes is very good for him but if he’s not spitting it back up, I can understand why you’d want to get it over with and get back to bed. If he goes on a pump at night, it might help getting him to take the bottle during the day because he won’t be used to getting it so fast. You might want to check and see if there is a feeding problems group in your area. Our local hospital has one. We haven’t been to it but I understand they discuss things like the problems you’re having getting him to take oral feedings and you might pick up some helpful ideas.

Mary Beth
MCAD mom

At school, the office secretary gets the feeding ready ~ attaches the formula bag to the pump and then Kyle plugs himself in and hits the start button. The school told us that “only a nurse” can administer the feeding so we got around that by teaching Kyle how to “plug in” and hit start. If there is a problem with the pump (beeping, etc) then the office staff problem-solves and if they can’t fix it, they call the school nurse. Actually, I’ve been sending Kyle’s feeding in gatorade so he is no longer using the pump at school. It has been a lot easier.

Michelle
SCAD mom

According to Public Law 94-142, the school system is supposed to provide the support needed for a child on a feeding pump. Medical problems are part of the child’s disabilities and the school system is responsible for providing a nurse etc. This may mean that your child will go to a school that is out of district but I would contact the Special Education Coordinator for your school system to discuss these matters as he/she is trained to explain and deal with these matters. Just because your child may not need to be in special education classes does not mean he/she does not qualify for special assistance. Medical needs are part of their learning needs. Your child’s medical needs must be met in order for him/her to learn in a least restrictive environment. Sometimes school systems get away with a lot more than they should with respect to providing for the medical needs of children. You are the best advocate for your child.

Beth Morley

I would strongly suggest a pump! And the only one I would recommend is the ZEVEX (link on the FOD website). Brett has one and wears it in a little backpack. Nobody knows that the backpack is actually a feeding pump! It is very cool looking. Then at night we hang the backpack on a pole (or screw the actual pump to a pole and hang the feeding bag on the pole) and fill him up! We have it figured out to go off at 3:30 am so when he wakes in the morning he is hungry for breakfast with the family.

This past week he has had a heavy-duty chest cold and refuses to eat anything. So, instead of being stressed out, we all went about our lives and he was fed 24 hours a day through his backpack.

It is expensive, so be prepared for a good fight. Zevex will provide you with a good sample letter to have your doctor rewrite and send to the insurance company. Our insurance company bought Brett one because I complained that the Kangaroo one they originally sent him was not suitable for a 2 yr old (it was too large and heavy for him to wear on his back). In regard to feeding through a tube, Brett vomits if it goes in too quickly. Also, I once remember a nutritionist telling me that feeding a child too quickly for a PROLONGED period of time can result to damage to the kidneys, but I think she meant setting a pump at too high of a rate on a daily basis.

Gina
VLCAD mom

Subject: Cornstarch Supplementation

We were told you can mix cornstarch with anything as long as you don’t cook it. Our son is two and weighs 29 pounds. Dr. Vockley has us give him three tablespoons before bed, and then we wake him at 1:30 a.m. to give him three more tablespoons. We do the cornstarch during the night in hopes to avoid an NG tube. We’ve had three doctors tell us NOT to cook cornstarch. The reason for using it is because it’s a complex carb and something is lost if you cook it. He just spent Wednesday in the hospital getting IV Glucose because of the stomach flu. Is this something that would have been unnecessary had he had an NG?

Debbie
unclassified FOD mom

I have also mixed the cornstarch with just a little bit of cold water and pushed it through my daughter’s tube. Of course it may not be too tasty to drink!

Lisa
VLCAD mom

My son took cornstarch since he was two years. I remember that doctors in Argentina recommended it when he was diagnosed at the age of one year and a half, but I could not believe in them. “Non cooked cornstarch? They are crazy,” I thought. When we met Dr. Roe at Baylor (then he was two years) and he confirmed “the non-cooked trick” then I believed it. Now that he is six years old I’ve been thinking in the possibility of taking him off cornstarch at night. Night is the only time he takes it. He eats very, very well, a very high carbo diet (I always think that as he becomes older he will HATE mashed potato, our schedule for meals are different from yours – we have dinner at 9 p.m., so he is having the dessert at 10 p.m.) and he wakes up early in the morning, so he never fasts more than 10 hours. Next week we have the annual appointment with his doctor and I will tell her what I think. Of course I won’t do anything wrong, but as I know her I know that her answer will be “NO.” So, I will invite her to join the list and to mail Dr. Roe. I think that in our case cornstarch is useful for example for the days he goes to karate or football classes, but not every day. I want to be sure that giving or taking off cornstarch won’t cause any problems.

Maria Martha
VLCAD mom

I was told by our metabolic nutritionist and Doctors to use it ONLY once a day (at bedtime) and uncooked. ARGO brand (although they don’t know why) works the best. I was also told that it was NOT given to children before one year of age (I can’t remember the exact reason why…something to do with one of the organs unable to process it???) But I do remember them saying that it is not good before one year of age. The amount should be determined by your specialist – it is based on age and weight.

Gina
VLCAD mom

They recommend not starting the cornstarch in children under a year of age because they feel that they lack the necessary pancreatic amylase to digest the cornstarch. However, trials have been done in children as young as seven or eight months with success. At our metabolic clinic, I recommend the use of uncooked cornstarch as it provides a more delayed release of glucose. We also recommend that it NOT be mixed with lemonade or in something very acidic as it affects the metabolism of the cornstarch. Joyce
RD at a Metabolic Clinic in CA

Our son gets four tablespoons of cornstarch in his six oz of milk before bed every night. He’s 21 months old and 30 pounds. It sounds like we’re on the high end compared to the amount everyone else is giving their children. But it’s working well for us and we’ve been giving him the cornstarch for more than a year (of course, in lesser amounts when he was smaller).

Lisa
MCAD mom

What I understand is that the cornstarch mixed with food or drink is a “slow release” source of energy. Unlike sweet drinks or cereals that are used quickly during activity, the cornstarch breaks down and the sugars are available or used at a slower rate. We asked our specialist about using cornstarch with our 5-year-old MCAD child. He said that it was not necessary at this time as long as she is eating well. She has no lasting complications from previous episodes. As she becomes older and more active (as in sports and sports training), he may recommend it in the future. As of now, we are not using cornstarch.

Melanie
MCAD mom

My son gets three tablespoons mixed in with his skim milk and chocolate syrup. He is 50 lbs. and four years old. He drinks approximately 10 ounces every night before bedtime.

Bob
MCAD dad

I give my daughter (LCHAD, age 4.5, weight 38 pounds) two tablespoons of cornstarch each night. What I do is take 3/4 to a full jar of Gerber cherry vanilla pudding, one teaspoon sugar and water to moisten the cornstarch. Chill in fridge and feed it to her at 8:30 p.m. She has her breakfast at 8 a.m. and her blood sugar is always in the upper 80s when she gets up in the morning. This is a good reading for her. She is full of energy and ready to go. After a full meal her blood sugar only goes up into the mid 90s. Never goes much higher then that. When she falls to around 77 I notice a difference in her and always know she needs some carbs for a pick-up.

Melissa
LCHAD mom

Grifinn takes her cornstarch before bed each night in nonfat milk with any of the following: Carnation Instant Breakfast, Nestle’s Chocolate Syrup, Nestle’s Strawberry Syrup. I also make her milkshakes with fat-free vanilla frozen yogurt and bananas or frozen blueberries. These go over really well. I tell her that her “Chocolate” (doesn’t matter what flavor it ACTUALLY is) is very important and that she has to drink it all, and for the most part, she does. I have to stir/shake the beverage frequently since the cornstarch settles to the bottom. Another trick is to use a straw because then she gets the more concentrated part of the drink from the bottom of the cup. If she doesn’t drink enough to satisfy me, once she’s asleep, I get one of the large (5 ml) medicine droppers and put it to her lips and she has the instinct to drink it while I squirt it in her mouth a bit at a time. It’s probably a good idea to wipe the teeth off a bit before putting the kiddo down for the night. I worry that all of the sugar/starch will do damage in her toothies.

Kelly
MCAD mom

Since Isabelle has started the cornstarch at night she is not as hungry and doesn’t eat as big of a snack. She takes 2½ Tbsp and she weighs 27 lbs.

Sharon
LCHAD mom

Subject: Morning Vomiting

I am sending this out to you to see if you have any ideas about why once or twice a week Adam vomits before going to school. No, I don’t think it is because he doesn’t like school. He isn’t sick either. He gets up, has a good breakfast ~ poached egg, toast with strawberry jam, fat free butter, and his bottle 6oz fat free milk, 3t MCT Oil and 1t Carnitine. Then as I drive him to school or before he gets out of the car (it’s only 1.2 miles there) he vomits up his breakfast! I thought it might be because he has 7-9t of cornstarch with his late night bottle at 10pm or so and maybe his stomach is too full at 8am still to deal with breakfast. I have cut out 2t of cornstarch from these late night bottles when he has to get up early and eat an early breakfast and bottle to try to prevent this but I guess it isn’t working. Have you heard about anyone other FODs doing this? It is very frustrating. Fortunately we keep airsick bags in the car so we don’t have a mess. Sometimes I take him home but he isn’t sick and has no fever. Any suggestions are welcome.

Valerie
LCHAD mom

My daughter vomited every day with every feeding (and the doctor thought I was nuts) until they determined she had a FOD (at 18 months old) and her diet was restricted to Tolerex. Before that she was on every formula on the market (and every one contains Coconut Oil which she reacts severely to). With every change of formula, it was just a matter of time (usually within 2 weeks) before she would start throwing up again. The doctors couldn’t figure it out either ~ I was the first to stop giving her those formulas and saw an immediate improvement. I only wish I had done it sooner.

You might start checking all the food labels and seeing if there might be a pattern with any type of fat. Coconut Oil comes in all kinds of foods, not just formulas. It’s an idea and sometimes that’s all we have. I had to change Miranda from Safflower Oil recently (she cant have MCT ~ that’s what she reacts to) to Grapeseed Oil because after 5 years, she started having seizures again a few weeks ago. It took many tries before getting to the Grapeseed Oil and getting her seizures stopped. Why after 5 years did she start reacting to the Safflower Oil? I would love for some doc to come along and give me the answers. But, the fact is, they don’t have them all either ~ at least not for her and I am left up to experimenting what works for her.

Cindy
Unclassified FOD mom

My son, Jimmy (13), has LCHAD. He vomited daily (usually several times a day) until he was about 9. He has had numerous episodes of lethargy requiring trips to the ER ~ all but two with normal blood sugars. Every time he responded dramatically to an IV with D10. I’ve heard lots of explanations for this ~ “these kids can be hypoglycemic at the cellular level,” “their blood glucose level may be normal, but they aren’t able to access the glucose” and “their bodies have responded by “dumping” all available glucose into the blood causing a temporary rise in blood glucose readings.” I don’t know what the right answer is but I do know that the IV with D10 has worked for him. Our protocol letter also states that an IV with D10 should be started right away in the ER regardless of blood glucose levels. We also carry around and keep on hand small 4oz. bottles of polycose liquid and a 60cc (2oz.) syringe. Sometimes when we see the signs of an episode starting, we can avoid the hospital. He takes it okay, and has never thrown it up. Also, as someone else had mentioned, my son vomits food that appears to be totally undigested even many hours after eating it.

Mary
LCHAD mom

My daughter was initially diagnosed with reflux and treated as such after initially presenting with multiple cyanotic episodes (turning blue) during her first 2 weeks of life. They put her on Propulsid to control it, but she continued to vomit at least one entire feed/day. At 2 months of age, she was diagnosed with a carnitine deficiency (major symptoms: vomiting and hypotonia, or low muscle tone). It is my understanding that vomiting goes hand in hand with many of the FODs. In the case of children with hypotonia, it definitely makes sense. The reason they vomit or have “reflux” is because the muscle at the top of the stomach is also hypotonic and doesn’t close sufficiently in many cases. Taylor’s reflux improved right along with her muscle tone once carnitine was started. At 8 months of age, she was off her reflux meds and no longer vomiting. However, I would also like to comment on the idea that it has to do with the type of fat in the diet. About 4 weeks later, the vomiting again started, though it was more like the “morning vomiting” described by the Mom who asked the original question. That vomiting improved after a formula change. At the time, she had been diagnosed with both milk and soy “allergies,” though allergy testing was negative. I now feel that it really wasn’t an allergy, but an intolerance to the type of fat in the formulas.

Dori
Undiagnosed FOD mom

Subject: Seizures

Ashley has been having seizures since her first episode of hypoglycemia due to her MCAD. She was in a coma for a few days and they think this was the reason for the seizures. She recently was put on Phenobarb for them but when the dose was given it made her terribly moody and too hyper. The Dr changed the dosage to see if this would help with her behavior but it was not enough to control the seizures (she had one and when we tested her Phenobarb level they were low). He decided we should wean her off and start Tegretol. She had her first dose yesterday along with her 15mg of Phenobarb and she seemed fine.played hard all day (almost too hyper for my liking) and she ate a great dinner.

Shortly after dinner we went to a store and she was running and jumping with her sisters when she stopped playing all of the sudden only to start staring into space. My 5 year old came to me and said Ashley is in a daze (oh how I hate to hear that one) so I had my daughter run out to the car to get the glucometer. Her sugars were 83, which is not cause for concern. I wonder though.do their sugars read normally during a seizure or is that not an accurate reading? Do I have to wait until it is over? Also, I wondered if my glucometer was cold from being in the car and will that alter an exact reading?? Just wondered if anyone had thoughts on that. My concern now is why she is having these seizures when there is no fever present and no hypoglycemia. Has anyone had these problems and what kinds of meds seemed to work for your child? I welcome any feedback.

Lisa
MCAD mom

Johnny was on Phenobarb when he was 3 and 4 years old and did great for the first year and then wow! He was Mr. Hyper. Never slept and became unbearable and uncontrollable. We then went to Dilantin and Tegretal. Before the Phenobarb he was on Depakote, which he couldn’t continue with because it depleted carnitine. He was also on Topamax, which didn’t work out too well at all. We are now having the most success with Primadone (Mysoline), which breaks down into 15% Phenobarb. We have always seen more seizures with the withdrawal of a drug even though we know to do it incredibly slowly. 00000 Johnny’s seizures could be triggered by lots of different things, but only the very worst ones were associated with hypoglycemia. He also always had very elevated blood sugars with a seizure so sometimes we take a blood sugar after the seizure and than 30 minutes later just to be sure the blood sugar wasn’t an issue. Hope things get better. I know how incredibly frustrating it is to try to figure out what’s going on with the seizures and the meds.

Sue
MCAD mom

I have a 6 yr old son with an undiagnosed FOD and a seizure disorder. He was a newborn with a hypoglycemic episode 44 hrs after birth with seizures. Ryan was seizure free for 18 months after the initial episode until he had a fever. Ryan had fever-induced seizures 6 weeks apart which warranted seizure medicine. The neurological seizures were explained to me that Ryan had an irritability in his brain which probably was a result of his initial hypoglycemic episode with a blood sugar of 0. His blood sugars were always normal or high with a seizure. Ryan became hypoglycemic even with a D10 IV after a seizure because of all the glucose energy used up only once. Ryan had hypoglycemic episodes with seizures during the age of 3-4.5 yrs which came on very quickly with or without illness. When he had any seizures I had to differentiate between neuro vs FOD seizures with a blood sugar.

In reference to car vs ambulance to the hospital—I always called 911. I live 20 miles away from the hospital. My pediatrician advises it.

Tegretol and Dilantin were used in treatment of my son’s seizures. They were both effective. Tegretol was stopped for side effects. Dilantin was used for 2½ yrs until Ryan had break-through seizures. Depakene has been used for almost a year without major problems. (* Be aware that that can deplete carnitine levels so a carnitine supplement most likely will be recommended).

Maria
Unclassified FOD

Subject: Emotional Distress

Q. I’d like to ask about something very sensitive. Since the possible diagnosis of FOD has been made, I’ve done a lot of research and reading. I am constantly worried that James will get ill and die. I am always on edge. I do “trial runs” in my imagination on what to do if he is ill or vomiting. Is this a ‘natural response’ that comes with the diagnosis, or am I being paranoid? I assume that this will get better with time. How can I relax? I’m afraid to send him to daycare or leave his side. I’m very sorry if this makes anyone sad. I debated for 3 days on whether or not to write, but I’m not sure who would understand any better than you folks.

Denise
Undiagnosed FOD mom

Denise, you are not paranoid! When we got Jamie’s diagnosis I was frantic. I barely slept and watched her sleep constantly always frightened she would slip away on me. Daycare is still difficult but I am close by and can be there in 5 minutes if need be and am comfortable with the knowledge that the daycare knows to call me if anything seems remotely wrong. It does get better though. Hang in there.

Velda
MCAD mom

Let me say that you are normal to worry so about your child. We worry when our children are healthy. I used to check my healthy children during the night all the time. It seemed I had an internal time clock and got up every few hours just to watch them breathe. Then along came Wendy (LCHAD). We didn’t even know she had anything wrong with her until she was 2½, but still I checked on her. Then when we realized something was wrong.yikes! I watched her breathe all the time. I don’t know how I am sane today (or am I?). At times I worried about her death. I still do, but know that I can do only so much. She’s 22 now and is never too surprised to wake up in the middle of the night to see me looking down on her. It’s just part of our life. It’ll be part of yours and become natural to worry and to worry about worrying. I worry about her death the same today that I always have. It hasn’t changed, but I have accepted it. I worry about mine too, because I just can’t leave her. I’m her only parent now. However, I try to focus on happy times, which are many, thank goodness.

I say it for all, when I say this group has been a Godsend. We understand each other as no one else can. Even our families and close friends can’t feel what we feel, and thank goodness for that, as we wouldn’t want them to have our exact feelings. But it is nice to know that when we ask the questions we do and talk about the things we do on this network, that we are not judged, but understood. Take care, and remember we are all here for you.

Mary
Adult LCHAD mom

I really believe that the emotional distress of an FOD really never leaves us. My daughter, Olivia, just turned 5 and has LCHAD. I always seem to worry about something with her. Is her blood sugar okay? Is she okay with the amount of fat (she only gets 7 to 8 grams per day) she ate for the day? Is she coming down with a virus? Did she get exposed to a virus? The list just goes on and on. I thought I might worry less as she got older but I worry just as much. I think it is because most of us almost lost are child and know what emotions we went through at the time of the episode and we never want to go through that again.

I have found that I have learned something with each illness that she has had. I now know when to go to hospital for the IV and don’t second-guess myself any longer. At one time it was should we go or should we hold out and see what happens? I don’t do that any longer. I have found out that each time I have made the decision to go to the hospital it has been the right one. So hang in there. We all have the similar stresses. Some days seem easy and some days don’t. But we just count are blessing that we still have our child and even with the added stress we would not trade them for anything! They are a true blessing and so are we for having them.

Melissa
LCHAD mom

I only wish you didn’t have to debate for 3 days to write about this. I think everyone of us knows the emotional distress and toll that our children’s illnesses bring. With Johnny being 13, I will say it gets easier or maybe it’s just that we are more used to dealing with the situations that come up. Also, children do get sick less as they get older and the germs don’t seem to spread as easily.

When Johnny was first diagnosed, I tried to read everything I could although there wasn’t much out there and I didn’t find the support group until 3 years later. I think this support group and having friends and family nearby to share the stress are the best support as well as a supportive pediatrician. We rarely leave Johnny under anyone’s care, except for school, but only because we haven’t found anyone to take care of him. What we do is either family activities together or my husband and I get out by ourselves separately. That is what really saves my sanity, is being able to get out with a friend for a movie or shopping, even if it’s only once every few months. Having a cell phone has made a big difference. You can get out and be contacted any time.

We were once told by a doctor that we had had a miracle so don’t ever let our guard down because we may not get a second one. That is some of the best advice I’ve ever gotten because it made me be conscious of the fact that treatment is full time and is preventive. It becomes a habit over time so that it doesn’t feel like you are always on guard. Living this way kept Johnny out of the hospital from the time he was 2 until he was 12 so I guess it worked. Hopefully time, information and the support group will help you through some of the tough spots and remember, there are lots of us out here to ‘listen.’

Sue
MCAD mom

I too worry about Henry, especially at this time of year when there are so many stomach viruses going around. He goes to preschool and stays with friends, etc but I have a cell phone and pager so I am always available. I tell everyone to call 911 or take him to TX Children’s Hospital if anything happens. We live five miles from the hospital. Henry has a medic alert bracelet and they have record of him at the hospital. These things make me feel better but I am sometimes still overwhelmed with the sense of possible emergency at any time. Our pediatrician is also very supportive. I think that all mothers worry but we all have some very good reasons to worry.

Betsy
Unclassified FOD mom

Please don’t worry about sharing your feelings with this group. As you can see by the responses you’ve gotten, that’s what we’re here for. We understand the stress and worry that these disorders cause better than anyone else.

In my own experience, the fear and worry were worse at first because you’ve just found out about it and read all of this scary info on it and seen all the “worst case scenarios.” It’s fresh in your mind and you need some time to assimilate the info. I was so overwhelmed at first. I’m a pretty paranoid mom to start with so this just really gave me some ammunition for my fears. The more time passed and the more milestones Ethan passed (like his first ear infection which did not lead to a hospital stay!!!) I’ve relaxed quite a bit. It’s not that I don’t worry anymore, but it’s not all consuming. You’ll be amazed at what you get used to! I don’t leave Ethan in the nursery at church anymore because every time we did he got sick. It just wasn’t worth the risk to me. Otherwise, his grandmothers love to baby sit and have gotten used to his special needs (having a cell phone makes leaving him in their care a lot easier for me!!) My husband and I have reached a stage that I can best describe as cautious optimism. We still worry, but not constantly, and we don’t let it rob our joy of having such wonderful sons as Ethan and Eddie. We both think we’ll breathe even easier when he gets to be 4 or 5. Something about that age seems less fragile then now. I’m sure once James is stabilized and diagnosed and into a routine, you’ll breathe easier.

I think it’s OK to worry enough to have a plan of action for what might occur, but once you’ve got the plan formulated, try not to dwell. Give yourself some time to recover from the bomb that’s been dropped into your life. Take all that emotional energy and let it express itself in love to James, but don’t be too hard on yourself. My sister told me something that really helped me. She said, “You need to give yourself the opportunity to grieve the loss of a healthy child.” We were blessed not to lose our precious baby, but nevertheless, our lives will never be the same. Don’t expect to be able to shake that off too easily. Soon enough you’ll adjust and take it more in stride. If you get the packet of info from Deb, there’s a story in it called “Welcome to Holland” that really helped me a lot. This isn’t what you expected but it’s not so bad. Try not to let this rob you of the joy that you still have James ~ that’s no small thing. The other thing that helps is to talk to other FOD parents. “Is this normal? Has your child ever had this happen?” It’s been so helpful to me to find out that there are other people out there who are functioning, even enjoying life with an FOD.

I guess what I’m trying to say in my overly wordy way is, it’s normal to be very worried now, it will get better with time, and we’re here for you if you need to talk.

Mary Beth and Ed
MCAD parents

You’re not being paranoid you’re being a parent. I’m extremely nearsighted, and I wore my glasses to bed at night for months because I wanted to be able to look over and see Katie if she made any sound or if I woke (assuming I ever got to sleep).

Gail
MCAD mom

I am glad to see some humor flow through. We all need that too! For six months I slept on the floor by Rachel’s crib so I could hear the ventilator alarm, when in fact it was so loud my neighbors could hear it. My back has never been the same!

Lisa
VLCAD mom

First, Denise, never feel like you can’t share with us WHATEVER it is that is on your mind. That is the only purpose of a support group! I think that thinking through possible scenarios in your mind and how best to handle them is a great idea. Then, once you come up with a good way to handle a possible crisis, then maybe you can relax knowing that if an emergency arrived you are prepared. I did this after my son was diagnosed and it helped me feel safer and more comfortable and more in control. Some things I did were:

Xerox 20 copies of your PROTOCOL letter and give one to everybody! Keep one in all your glove compartments, diaper bags, etc. Reduce one on your computer for your partner’s wallet. Even my local doctor carries one in his wallet! This was in case he gets a 3:00 am phone call from me, he can reach into his wallet and know what to tell the ER (I think it makes him feel more secure too!)
Have your local doctor educate everyone in his/her practice about your child. In case he/she is unreachable, the covering doctor needs to have background info. Even the nurses and receptionist in my local doctor’s office know that if I call I am to be given no grief and seen whenever I want to be seen. They are wonderful and know that Brett can quickly go downhill from even a little cold so they try very hard to accommodate me. However, they would not know this if the doctor didn’t speak to them and tell them Brett’s story.
Your local doctor needs to speak with the ER staff of the hospital he/she is affiliated with. They can keep your child’s info on record.
Go online to Medic Alert (link on FOD site under Sites of Interest) and order bracelet for your child. They have the info from Brett’s protocol letter on file and can fax it to any ER if needed. I also feel that when that bracelet is on, the ER admitting nurse takes me more seriously. The back of the bracelet says “Rare metabolic disorder. Subject to CHF and Coma” (CHF means congestive heart failure). It seems a little dramatic, but gets people moving!
Pack an over night bag and keep it in the back of your closet. You might never need it, but if you do need to sleep over in the ER, you will be grateful you planned ahead! Have a copy of insurance card in it as well as money, change, phone numbers, toys, snacks for you, etc.

This sounds simplistic, but try to relax and enjoy your child. You will look back 15 years while sitting at the high school stadium watching him/her hit a grand slam home run and regret that you worried his/her childhood away. They grow up in a wink of an eye. Don’t let this disorder rob you of these precious years.

Gina
VLCAD mom

Thank you so much to everyone who’s responded and to those who deal with this everyday. You have done so much to help me. I feel like I can face this now. I’ve printed out your responses for those days that are difficult. I hope they are few and far between. I can’t express in words how helpful it is to know all of you are out there. I’ve been praying for answers and a diagnosis for 4½ years, now I’ll have to take what comes. Bless you all.

Denise
Undiagnosed FOD mom

Subject: CPK Levels

This morning Isa had a real bad episode. She awoke with a blood sugar of 29. After treatment it went up to 42 (15 minutes). Following more treatment and arrival at the doctor’s it was 203. While we were there our pediatrician showed us the letter that our metabolic specialist had sent her. In the letter it said that Isa’s FOD is a possible VLCAD (later changed to LCHAD). Also the letter indicated that at her last doctor’s appointment she had elevated CPK levels. What is CPK?

Sharon
LCHAD mom

CPK stands for creatine (phospho) kinase, sometimes also referred to as “c k” level. It measures enzymes in the blood from muscle breakdown and there is an extended cpk that detects isoenzymes that tells if it is coming from heart muscle or skeletal muscle. Rachel’s has always been totally muscular, although if her body is not getting what it wants and enough of it (high cpk alerts me to this), it can be very damaging to her heart since this is one of the effects of the particular phenotype of the disease that she has. We routinely check her CPK when she is sick. With illness or infection the metabolic rate speeds up and with VLCAD, inadequate calories can lead to muscle breakdown. We also have checked it when she is well to see what her “normal” average level runs (lab norm 0-225).

Lisa
VLCAD mom

Our kids’ CPKs rise every time they get an illness. It was explained to me in laymen’s terms like this: Your body is like an engine. It gives off exhaust just like an engine. The CPK level is the level of exhaust caused by the breakdown of muscle in your body. Normal levels for a toddler is 50-300. When an FOD child gets ill, they use all the sugar fuel first then reverts to the breakdown of skeletal muscle for energy. This causes the rise in the CPK (exhaust) levels.

Justin’s high has been 87,000. Autumn’s high 39,000. They always go back to normal. Originally the heart was a concern, but we have since ruled that out since the breakdown is from their skeletal muscles only. They treat it with sodium bicarbonate IV. I think the key to keeping the CPK levels down is keeping the sugar levels high and increasing the fluids. The main concern of high CPK levels is kidney damage. The kidneys are responsible for cleaning the blood and if there is a lot more “exhaust” in the blood it really puts a strain on the kidneys and can cause some permanent damage. This may not be technically correct in all aspects, but that is how it was explained to us.

Kim
LCHAD mom x 2