12 January 2004

1. Neurofibromatosis
2. Nursing and Seizure Meds
3. Toxicity of Clorox
4. Tracheoesophageal Fistula
5. Hazards of Peanut Butter
6. Infant Vomiting
7. Tummy Ache
8. Elevated PTT
9. Disclaimer

• Read Questions & Answers in Another Column

Neurofibromatosis

Thank you.

-Holly

Dear Holly: The following information regarding Neurofibromatosis is from MD Consult Online, Copyright © Clinical Reference Systems 1999 Pediatric Advisor. If this is not the condition you are seeking information about, check the spelling and get back to me. This was the closest I could come to neurobromoosis

Description

Neurofibromatosis 1 is also called generalized neurofibromatosis or NF-1. It is a genetic and chronic (ongoing) condition affecting about 1 in 4000 people.

Nearly all children with NF-1 eventually have:

• large brown skin spots called cafe-au-lait spots (Most people with NF-1 have six or more cafe-au-lait spots. Fewer than six spots is fairly common in people who do not have NF-1.)
• neurofibromas (noncancerous tumors of the sheaths of the nerves) in the skin or elsewhere in the body.

Parts of the body that may be affected include the eye, the bones, and sometimes the blood vessels, gastrointestinal tract, nerves, and brain.

Diagnosis

NF-1 is diagnosed when two or more of the following features are present:

• several cafe-au-lait spots
• two or more rubbery bumps called neurofibromas
• freckling in the armpits or groin areas
• a growth on the nerve to the eye called an optic glioma
• growths on the iris of the eyes called Lisch nodules
• thinning of the long bones (with or without joint problems)
• a first-degree relative (parent, brother or sister, or child) with NF-1.

Growth and Development

Infants with NF-1 tend to be shorter than average, with heads somewhat larger than average.

When a child with NF-1 is born, he or she may have only the cafe-au-lait spots. The size of the spots varies from 1/4 inch in diameter to several inches. Sometimes newborns have armpit freckling and occasionally neurofibromas.

New cafe-au-lait spots often appear during infancy and early childhood; the spots do not harm your child. As children grow older, the cafe-au-lait spots and neurofibromas tend to increase in number and size. Neurofibromas are particularly prone to increase in size during adolescence and pregnancy.

Neurofibromas may develop in other body organs besides the skin. Sometimes they cause problems, depending on where they develop. Neurofibromas can put pressure on vital structures (for example, blood vessels) and thus do damage to the organs they are pressing on. Neurofibromas need to be removed surgically when they:

• may damage vital organs
• cause pain
• are infected
• are a cosmetic concern.

For reasons that are not well understood, learning disorders are more common in people with NF-1 than in the general population. Speech problems, hyperactivity, attention problems, seizures, and mental retardation are also somewhat more common. High blood pressure may occur. Some cancers occur in greater than expected rates in persons with NF-1.

Health Care for a Child with Neurofibromatosis

Many doctors care for a child with NF-1. The schedule for well-child visits is the same as for other children, but there are some extra services and care your child needs.

• Infancy: Regular check-ups, including blood pressure measurements, are important. Be sure to cover your child with sunscreen when he or she is out in the sunlight.
• As parents of a newborn with NF-1, you may wish to discuss with a geneticist concerns you have about your child or other family members and the possibility of having other children with NF-1.
• 1 to 4 Years: Neurofibromas of the skin may develop during these years and may require medication to prevent itching. It is important for your child to have an annual eye exam by an eye doctor and a hearing exam before he or she enters preschool or kindergarten.
• 5 to 12 Years: Some children with NF-1 enter puberty (sexual development) too early. Your doctor will check your child for pubic hair, breast enlargement, or other changes that suggest sexual maturation at an age that is too young. Your doctor may also review your child's school achievement and ability to get along with others.
• 13 to 21 Years: Curvature of the spine, known as scoliosis, occasionally affects adolescents with NF-1 and should be checked for periodically.

The best treatment for neurofibromas is not yet known but many studies are in progress to determine if they can be reduced.

Call Your Child's Physician During Office Hours If:

• You are worried about your child's:
  • learning or behavior
  • vision
  • nervous system
  • curvature of the back.
• Benadryl does not relieve the discomfort from itching.
• You have other questions.

Additional Resources

The National Neurofibromatosis Foundation (800-323-7938) provides information about medical concerns as well as resources in the community for early detection of problems and support groups. The NNFF web page can be accessed at http://www.nforg/.

A book for parents is:
Medical Publishers, 1990.

Sincerely,
Dr. Warren

-DI

Dear DI: Tegretol is secreted in human milk but the concentrations in the blood of nursing infants is not too high and there have been no reported problems related to nursing while taking Tegretol. Neurontin is a relatively new drug. It is not known whether or not it is secreted in breast milk. No studies have been done regarding the safety of Neurontin in nursing mothers. If the amount of your milk the infant gets is minimal, logic suggests that there shouldn't be any problem, but where there is lack of knowledge and potential for problems, I can only urge caution.

Sincerely,
Dr. Warren

-a Nursing Student

Dear Nursing Student: Your classmate is correct. According to Ellenhorn's Medical Toxicology, 2nd ed., Copyright © 1997 Williams & Wilkins, Most household bleaches (e.g., Clorox) contain less than 5% sodium hypochlorite, which causes a moderate mucosal irritation. This could produce nausea, vomiting, or abdominal pain. It would require a massive ingestion to produce toxicity. The concentration of sodium hypochlorite in Clorox is not high enough to be caustic. Industrial strength bleaches and granules which are a higher concentration could be caustic.

Sincerely,
Dr. Warren

Thank you so much for your help.

-DM

Dear DM: The following information comes from National Organization for Rare Disorders, Inc.(NORD)
55 Kenosia Avenue
PO Box 1968
Danbury, CT 06813-1968

Phone Number: (203) 744-0100
Tollfree: (800) 999-6673 (voicemail only)
TDD Number: (203) 797-9590
Fax Number: (203)798-2291
E-mail Contact: orphan@rarediseases.org
Home page: http://www.rarediseases.org/

Copyright 1992, 2000

Synonyms of Esophageal Atresia and/or Tracheoesophageal Fistula

* Atresia of Esophagus with or without Tracheoesophageal Fistula
* Esophageal Atresia
* Tracheoesophageal Fistula
* Tracheoesophageal Fistula with or without Esophageal Atresia

General Discussion

Esophageal Atresia and Tracheoesophageal Fistula are disorders of the esophagus that may be inherited as an autosomal recessive genetic trait, or may result from developmental problems in a fetus. Esophageal Atresia is a condition in which the patient is born with an abnormality in the part of the digestive tube that runs from below the tongue to the stomach (esophagus). This disorder is commonly associated with Tracheoesophageal Fistula which is an abnormal tubelike passage between the windpipe and esophagus. Symptoms of these disorders may be excessive about salivation, choking, the return of swallowed food into the mouth, and/or a swollen abdomen when a Tracheoesophageal Fistula is present.

Organizations related to Esophageal Atresia and/or Tracheoesophageal Fistula

• EA/TEF Child and Family Support Connection, Inc.
  111 West Jackson Boulevard
  Chicago IL 60604-3502
  Phone #: 312-987-9085
  800 #: --
  e-mail: info@eatef.org
  Home page: http://www.eatef.org
• NIH/National Digestive Diseases Information Clearinghouse
  2 Information Way
  Bethesda MD 20892-3570
  Phone #: 301-654-3810
  800 #: 800-891-5389
  e-mail: nddic@info.niddk.nih.gov
  Home page: http://www.niddk.nih.gov

• TEF/VATER/VACTRL National Support Network
  15301 Grey Fox Road
  Upper Marlboro MD 20772
  Phone #: 301-952-6837
  800 #: --
  e-mail: tefvater@ix.netcom.com
  Home page: http://www.tefvater.org

Sincerely,
Dr. Warren

Thanks.

-Steve

Dear Steve: Peanut butter is a choking hazard just like peanuts because it is sticky. Soft bread with peanut butter can easily get caught in the roof of a child's mouth and be difficult for a young child to manipulate. Peanuts are also a potent allergen. Peanut butter carries the same allergy risk as peanuts. It is generally recommended that peanut butter be avoided until a child is at least 2 years old.

Sincerely,
Dr. Warren

Thanks

-JAM

Dear JAM: There are so many possibilities for the 5 week old who appears to be vomiting, that you will need your pediatrician's help to sort it out. As long as the baby is holding down most of his feedings and the vomiting does not escalate in frequency or amount, your son is in no danger. The main risk of vomiting is dehydration. If your son is urinating well and feeding well, you have time to work with your pediatrician to figure this out.

Many babies appear to be vomiting when they are really just spitting. Big spitters may not start out that way because they take small feedings as newborns. As the volume of their feedings increases they start to bring up formula after each feeding, often large amounts with burps and smaller amounts in between. Your pediatrician can help determine if that's what's going on with your baby by following his weight gain and determining if he is thriving.

If the baby starts to have projectile vomiting (vomiting that shoots out forcefully), at 5 weeks you have to think of pyloric stenosis. If the baby is not gaining well, has projectile vomiting and vomiting after most feedings, your pediatrician can get an ultrasound study to look at the pylorus. It might also help for your doctor to observe a feeding and see the vomiting.

If the baby continues to vomit and has a great degree of discomfort, he may be having a significant amount of gastroesophageal reflux. These babies may gain well, but they are irritable from the discomfort and may have coughing or wheezing. If that is the case, an evaluation by a gastroenterologist can help determine if medication would be useful.

Formula sensitivity can cause vomiting. Since soy is not a hypoallergenic formula, it might be better to try the baby on a formula like Nutramigen or Alimentum.

Sincerely,
Dr. Warren

Before returning to work his stool was full of curds and his bowel movements occured about 5 times a day. I started drinking chamomile tea and hoped that since I'm breastfeeding him, he could somehow feel the stomach calming effects of this tea. I've also been giving him MyIicon drops when his tummy aches occur.

The result is that his tummy aches are less frequent but still occuring and his stool is still completely liquid.

My question is: Do you have any suggestions of anything else I could try?

-EPK

Dear EPK: From your description, I am not sure that anything unusual is going on. If I understand correctly, your baby is having bowel movements every 3 days. Even if they are watery, since they are infrequent, this does not represent diarrhea. Many nursing babies go from a bowel movement after every feeding to a BM every 3 to 5 days around 3 to 5 months of age. Your baby may just be following the natural course of events which just coincidentally coincide with your return to work. Other factors which may play a role include changes in your diet due to being back at work and swallowed air resulting from bottle feedings. These may be improved by altering your diet and experimenting with different bottles.

It is also possible, with your being back to work, that you brought home an intestinal virus without being too sick yourself. If your baby has an intestinal virus, it is probably on its way to resolution by now. For more information about intestinal viruses, read my article, Management of Gastroenteritis.

Sincerely,
Dr. Warren

-DR

Dear DR: An elevated PTT indicates an abnormality in the normal clotting mechanism such as a deficiency of one of the clotting factors. Your granddaughter should be evaluated by a hematologist prior to having surgery. She may require treatment with clotting factors prior to surgery.

Sincerely,
Dr. Warren

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