Acyl-coa dehydrogenase, very long-chain, deficiency of

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How do I know when to get medical care for my child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD)?

You should discuss with your metabolic doctor and metabolic dietitian about what to do when you are concerned about your child's blood sugar during your initial visit and during subsequent visits. Over time, you will get to know your child and the best way to respond when they are not eating due to illness or stress. You will also learn the signs that indicate you should take your child for emergency treatment.

If you child is sick, it is a good idea to feed them more frequently and to contact your metabolic doctor.

When you first notice that your child is not eating, you should offer him/her a favorite high sugar food, such as fruit juice. It is best to avoid Pedialyte and other rehydration drinks which do not have adequate amounts of sugar to help a child with VLCADD recover once their blood sugar starts to drop.

If a child continues to not eat, is vomiting and/or if they show signs such as low energy (lethargy) or irritability, take them to an emergency room for evaluation and treatment. Take your child's emergency protocol letter with you and present it on arrival.

A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website. If you are looking for a metabolic clinic for treatment in the United States, you can search for the clinic or clinics in your state.

A metabolic dietician can be found through the Genetic Metabolic Dieticians International webpage.

A genetic professional can also be found on the Genetic Testing Registry Homepage.

References
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What is an emergency protocol letter?

How do I get an emergency protocol letter?

What should an emergency protocol letter for a child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) include?

What do I do if I or my child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) needs surgery?

What should I do when my baby with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) is beginning to sleep longer without eating?

Are there any things that people with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) should avoid?

Can a child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) grow up and live a healthy and productive life?

Can I buy carnitine supplements at the health food store to treat very long-chain acyl-coa dehydrogenase deficiency (VLCADD)?

What is an emergency protocol letter?

Emergency protocol letters provide information about your/your child's diagnosis of Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) and give specific treatment instructions to health care providers in emergency rooms or other health care settings that are not familiar with the diagnosis of VLCADD. Because children with VLCADD can have their blood sugar levels drop quickly to dangerously low levels when they become ill, they often require intravenous (IV) glucose in situations when a child without VLCADD would recover on their own without treatment. If healthcare providers are not familiar with you/your child and the diagnosis of VLCADD, there may be a delay in treatment.

The emergency protocol letter will provide the necessary information to explain the diagnosis and correct treatments so that a health care provider not familiar with VLCADD will be able to provide the correct treatment for a person with VLCADD as quickly as possible.

Up to date protocol letters should be carried at all times with the family or caregiver of the child. Many people have found it helpful to keep the letter in a plastic folder or bag in the child's diaper bag. It may be necessary to have multiple copies of the letter to be able to have them available to all parents and caregivers. It may also be helpful keep copies of the letter at local hospitals, but because these can take time to access, a hard copy of the letter should also be with the child.

Speak with your metabolic physician to develop an emergency protocol letter.

References
How do I get an emergency protocol letter?

Your metabolic geneticist will provide the letter. The letter should be reviewed and updated regularly. Each time the letter is updated, make sure to any destroy previous copies of the letter and replace them with the new version. Speak with your metabolic physician to develop an emergency protocol letter.

References
What should an emergency protocol letter for a child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) include?

The letter typically includes:

  • Your/your child's name and date of birth
  • A brief summary of your child's medical history
  • A statement that the child has VLCADD and a brief explanation of the condition
  • Specific instructions for testing and treatment when you/your child has symptoms that may put them at risk for dangerously low blood sugar levels. These instructions will be unique to you/your child
  • Your metabolic geneticist's emergency phone (which should be available 24 hours a day, 7 days a week) and your primary physicians phone number
  • Your doctor's signature

Speak to your metabolic physician to develop an emergency room letter.

References
What do I do if I or my child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) needs surgery?

If you child will need to go without eating because of surgery, have the surgeon work with your metabolic physician to plan the best way to prepare for surgery and guarantee that you/ your child receives adequate nutrition before and during surgery. Sometimes, individuals with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) are admitted to the hospital the night before surgery.

References
What should I do when my baby with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) is beginning to sleep longer without eating?

Speak to your metabolic physician and plan ahead for when your baby will be sleeping longer at night. Initially, you may be told to wake your baby frequently to feed him/her. As he/she gets older, you can may be able to let him or her sleep longer unless they are sick. Your doctor may also give you instructions about certain kinds of foods or supplements such as medium chain triglyceride (MCT) oil or cornstarch that may help a sleeping child go longer without food.

During this phase it is important that you stay in touch with your metabolic physician and adjust your child's sleep schedule and feeding to avoid long periods without eating.

References
Are there any things that people with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) should avoid?

People with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) may need to avoid the following situations:

  • Going long periods without food
  • Becoming dehydrated
  • High fat diets, including ketogenic diets and low-carbohydrate diets for weight loss
  • Strenuous exercise the results in muscle breakdown
  • People with the severe forms of VLCADD may need to avoid certain heart procedures such as cardiac catheterization which may irritate the heart muscle.

Please consult your metabolic physician to determine which of these apply to your situation.

References
  • Leslie ND, Valencia CA, Strauss AW, et al. Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency. 2009 May 28 [Updated 2014 Sep 11]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. http://www.ncbi.nlm.nih.gov/books/NBK6816/
Can a child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) grow up and live a healthy and productive life?

With proper treatment, a child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) can grow up to be healthy. Newborn screening and early diagnosis are important to preventing complications. It is important that the parents and caregivers are aware of the risks of low blood sugar when a child goes a long time without eating or has increased calorie needs. As the child grows up, he or she will begin to recognize when he/she is at risk for getting low blood sugar and will be able to take more control of their treatment and be able to ask to get treatment with intravenous (IV) sugar. Speak with your child's pediatrician and metabolic physician if you have concerns about their growth or development.

References
Can I buy carnitine supplements at the health food store to treat very long-chain acyl-coa dehydrogenase deficiency (VLCADD)?

If you are being treated for very long-chain acyl-coa dehydrogenase deficiency (VLCADD), your doctor may recommend you take carnitine. Although there is a version of this supplement that you can buy at health food supplement stores, the carnitine there has not undergone the amount of rigorous testing and regulations that the prescription versions (Carnitor® and levocarnitine) have. In fact, some supplements bought from the health food store are not manufactured in the United States. Since you do not know for sure the quality of the product, it is recommended you discuss use of this supplement from the health food store with your doctor. The safest alternative is a medication that has been approved and is regulated by the Food and Drug Administration to treat VLCADD.

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