500th Case Milestone

This past June is a special month for PediatricEducation.org. We published our 500th case!

It has been an amazing journey that started with just one weekly case and now we have come to this milestone.

We cannot have done it without you our patrons, so we thank you for all of your feedback and support. A very special, thank you to each of you.

Donna M. D’Alessandro, M.D. and Michael P. D’Alessandro, M.D.
Curators, PediatricEducation.org

What Does Discipline Really Mean?

Patient Presentation
A 3-year-old male came to clinic for his health supervision visit. He was generally well, but his behavior was becoming more problematic after his sister’s recent birth. He would hit his parents, hit or pinch the infant, throw toys and food, refuse reasonable requests such as dressing, brushing teeth, leaving a room or the home. He was also coming into his parents bed at night which they did not like but said they didn’t want to fight during the night as they were so fatigued. “It’s just a struggle all the time,” his mother lamented. “He’s never been easy and always has been a challenge.” The child has always been described as having “high energy” and had crying and colic as an infant. He often had problems with sleeping because of frequent infections and his mother had found it difficult to be consistent with his behavior. She said she knew to use time outs to discipline him but when he refused to move into time out she often gave up. She denied hitting the child but found herself often yelling at him. The past medical history shows a former 36 week infant with multiple episodes of otitis media requiring pressure equalizing tubes at 15 months. The family history was negative. The social history shows that mother was a homemaker and the father worked evening and night shifts. They had no family in the area. The mother and son would have play dates with other friends once a week. The review of systems was negative.

The pertinent physical exam showed a well-appearing male with growth parameters in the 25-75% and had normal vital signs. His examination was normal but he was highly verbal and resistive to the physical examination in the room. He refused to comply with simple commands until asked several times and then would comply. He put books and crayons around the room and refused to pick them up. He often seemed to be looking for approval from the mother and pediatrician. The diagnosis of a healthy but non-compliant 3 year old was made. The pediatrician talked with the mother about the reasons for discipline and strategized ways to consistently implement time out. “His behavior is certainly going to get worse before it gets better, but once you start doing this consistently he will know exactly what the rules are,” the pediatrician said. “He’s a good boy who wants to know what the rules are and how to behave. He also wants your attention, so you need to give him attention when he is doing things well. A pat on the head or shoulder, a kind phrase goes a long way to having more of the good behavior happen.” He recommended using a reward system such as stickers or time with a parent for good behavior. “He has a really good vocabulary, so you can use his verbal strength to talk with him about what rules should be set up and then how to follow them,” recommended the pediatrician.

Discussion
Being consistent, setting limits and giving choices and guidance are keys to effective discipline. “Children are not born behaving according to societal norms and complying with their parents wishes; it is the parents role to teach the child how to behave cooperatively with others.”

The word discipline comes from the word disciple which means to teach. There are three things for parents to discipline their child over that are non-negotiable which are issues of health, issues of safety, and issues of their future. For example, a child cannot run into the street or ride in the car when someone has been drinking. A child needs to go to school so they can be successful in life. But having a messy room is not a health, safety, or future issue and therefore can be negotiated with the family.

Corporal punishment is normative in many cultures. Parental use of physical punishment or maltreatment is most often not from the desire to harm the child but from the intent to punish or to teach the child. Corporal punishment however increases the risk of physical injury and children have more aggressive or agitated behaviors over the long-term. Corporal punishment may also cause the escalation of the behavior by caregivers. The 1999 World Health Organization Consultation on Child Abuse uses the following definition “child abuse or maltreatment constitutes all forms of physical and/or emotional ill-treatment, sexual abuse, neglect or negligent treatment or commercial or other exploitation, resulting in actual or potential harm to the child’s health, survival, development or dignity in the context of a relationship of responsibility, trust or power.”

There are different parenting styles often characterized by the amount of control and support the child receives.

  • An authoritarian parenting style is one where parents are very controlling of the situation and provide little support. The parent sets the rules with little discussion or explanation, and this provides less support for the child’s own emotional growth and independence. Use of shaming and corporal punishment is common for the child’s noncompliance.
  • Permissive parents provide little control, limit setting and little support. Children are often described as “running wild”. For non-compliance, the child may receive no discipline or it is ineffective or inconsistent. The child is left to learn for themselves.
  • Authoritative parenting style is one where parents set defined limits but teach the reasons for those limits and provide correction in a consistent manner. The discipline is meant to enforce the rules yet teach the reasons for the rules. Authoritative parents are more supportive of their children’s risk-taking yet provide strict limits and guidance for the behaviors that are expected.

Learning Point
Children of all ages, just like adults, want to know what the expectations are, want to know that they are doing a good job in their work or play (i.e. affirmation), and to have choices in their lives. Naturally children have fewer choices than adults as adults must necessarily limit children’s choices to provide a safe environment. At the same time parents need to allow children to learn to make good choices and avoid risks inherent in life. In a simple example of getting dressed in the morning a parent can offer the child the choice of putting their shirt or pants on first. This sets the expectation that the child will get dressed. Either choice complies because the parent just wants the child dressed. Finally, the child receives affirmation that they have done well by getting dressed. Both the child and the parent win in this situation as the parent has set the child up to win. Obviously choices cannot be given all the time because of time constraints, resources and the specific situation.

Consistency is the key for parenting. The parent can set consistent expectations and limits for the child, then the child will learn and know what to do in the same or similar situations over time. For example, a parent can remind the child to hold onto a shopping cart when walking in a store. When the child does not, the parent can remind the child again and when the child continues to hold onto the cart can give brief praise for doing so. In new situations, the child can try to transfer that learning to the new situation, such as going to a market or entertainment venue. The parent can say that this new place is different but the same rule of holding onto the stroller or a parent’s hand is the same. A parent that sets expectations and acts consistently makes it much easier for the child to understand the situation and to be able to comply.

“Parents can encourage [healthy children] by creating a loving, supportive, and caring environment and by setting appropriate limits and boundaries for their children.” Teaching discipline to parents is an important part of anticipatory guidance. This is usually done by verbally giving advice and providing written materials for families. Use of multimedia technology such as videos or interactive technologies can be helpful. Using electronic health record portals, electronic mail, cellphone messaging and social media can also help to educate and support families as well as reinforcing positive discipline techniques.

Questions for Further Discussion
1. What were common discipline techniques used in your home as a child? How did those affect your life and your professional education of families?
2. What are common discipline techniques used by families in your community? How does culture affect those techniques?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Child Behavior Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Dictionary.com. Disciple. Available from the Internet at http://dictionary.reference.com/browse/disciple?s=t (cited 5/7/15).

Committee On Psychosocial Aspects of Child and Family Health. Guidance for effective discipline. Pediatrics. 1998;101:723-8.

Owen DJ, Slep AM, Heyman RE. The effect of praise, positive nonverbal response, reprimand, and negative nonverbal response on child compliance: a systematic review. Clin Child Fam Psychol Rev. 2012 Dec;15(4):364-85.

Krug EG et al., eds. World report on violence and health. Geneva, World Health Organization, 2002. p. 195.

Kolhatkar G, Berkowitz C. Cultural considerations and child maltreatment: in search of universal principles. Pediatr Clin North Am. 2014 Oct;61(5):1007-22.

Glascoe FP, Trimm F. Brief approaches to developmental-behavioral promotion in primary care: updates on methods and technology. Pediatrics. 2014 May;133(5):884-97.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital

How Does Spinal Muscle Atrophy Present?

Patient Presentation
A 4-month-old male came to clinic for his well child examination. His mother’s only concern was when he should be taking solid food. He was smiling, cooing and could roll from front to back when laid prone. The past medical history showed a former 36 week premature infant because of maternal chrorioamnionitis. Initially he had temperature instability for several hours and mild hypotonia. His initial screening and repeated laboratory testing were negative, but he received presumptive antibiotics for 5 days because of the temperature instability and hypotonia.

The pertinent physical exam revealed a smiling infant with growth parameters in the 25-50% with normal vital signs. His examination was normal with normal tone and strength. He still had a small head lag when pulled to sitting from a recumbent position. The diagnosis of a healthy male was made. The pediatrician discussed not to start solid foods currently as the child still had a head lag but explained that this was normal even for a child who was not born prematurely. After the visit the medical student who was following the pediatrician asked several questions about the differential diagnosis of hypotonia. The pediatrician explained that the most common cause at birth was something that could usually be fixed fairly quickly such as an electrolyte problem or presumed sepsis, but that other causes were possible. She asked about some genetic causes and wanted to know when spinal muscle atrophy presented. The pediatrician discussed that it could start quite early and could be very aggressive in its increasing weakness or that it could be much milder. “I’ve only taken care of a few patients and they were infants when they were diagnosed. They had had severe disease and many problems” he explained.

Discussion
Muscle tone is the slight tension that is felt in a muscle when it is voluntarily relaxed. It can be assessed by asking the patient to relax and then taking the muscles through a range of motion such as moving the wrists, forearm and upper arm. Muscle strength is the muscle’s force against active resistance. Impaired strength is called weakness or paresis. There are 5 levels of muscle strength. Hypotonia can occur with or without weakness. Decreased fetal movements in utero, persistent hypotonia and difficulty feeding are more consistent with congenital rather than an acquired hypotonia. Common treatable conditions such as hypothyroidism, electrolyte abnormalities and metabolic problems can often be ruled in or out relatively quickly. A differential diagnosis of hypotonia with and without muscle weakness can be found here. A differential diagnosis of hypotonia in infants can be found here.

Learning Point
Spinal muscle atrophy (SMA) was first described in the 19th century and is an autosomal dominant neuromuscular disease caused by a deletion or mutation in the motor neuron 1 gene on chromosome 5q13. It causes anterior horn cell loss in the spinal column and brain stem with progressive symmetric proximal muscle weakness. Patients also present with respiratory and orthopaedic problems. Hypotonia, decreased or absent deep tendon reflexes, muscle fasciculations and muscle contractions can also occur.

The gene frequency is 1 in 40-70 in the general population but the disease is less common than expected (varies 1:6000 to 1:25,000 births) most likely due to fetal loss or very mild forms that are undiagnosed. SMA1 gene is deleted in 95% of patients so a functional protein is not made. SMA2 is a modifier gene that has protective effects on the protein but is variable in its actions. SMA is detected by genetic testing. DNA assays of dried blood spots are possible making SMA a candidate for newborn screening.

There is no definite cure or specific treatment. Some treatments attempt to upregulate the SMA2 gene or to produce more protein such as valproic acid or hydroxyurea. Neuroprotective medications, such as gabapentin, are being evaluated Gene and cell therapy are also being studied.

SMA is usually classified by the age of onset of the muscle weakness which correlates with the progression.

  • SMA 0
    • Present at birth with severe weakness, respiratory compromise or failure and arthrogryposis
    • Death usually occurs soon after birth if respiratory support is not available.
  • SMA I
    • Also known as Werdnig-Hoffman Syndrome
    • Presents < 6 months of age
    • Severe muscle weakness and respiratory failure
    • Never sits independently and never walks
    • Life expectancy is < 2 years
  • SMA II
    • Presents < 18 months of age
    • Able to sit unsupported at some time but never able to walk
    • Life expectancy is 10-40 years
  • SMA III
    • Also known as Kugelberg-Welander Disease
    • Presents > 18 months of age
    • Able to walk at some point but usually require wheelchair assistance later.
      Life expectancy is indefinite
  • SMA IV
    • Onset of mild symptoms in adulthood
    • Sits and walks normally
    • Life expectancy is indefinite

Questions for Further Discussion
1. What is the role of genetic counseling for a family who are possible carriers?
2. For a child with Type III SMA, what are factors that help determine when a child should be placed into a wheelchair?
3. What other chronic health problems can children with SMA have?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Muscular Dystrophy

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Viollet L, Melki J. Spinal muscular atrophies. Handb Clin Neurol. 2013;113:1395-411.

Singh P, Liew WK, Darras BT. Current advances in drug development in spinal muscular atrophy. Curr Opin Pediatr. 2013 Dec;25(6):682-8.

Scully MA, Farrell PM, Ciafaloni E, Griggs RC, Kwon JM. Cystic fibrosis newborn screening: a model for neuromuscular disease screening? Ann Neurol. 2015 Feb;77(2):189-97.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital