A child has been diagnosed with fragile X syndrome. The nurse would predict that the child may exhibit which of the following signs/symptoms?
Strabismus
Arm flapping
Vision deficit
Nevus flammeus
The Correct Answer is B
Fragile X Syndrome (FXS) is caused by a mutation in the FMR1 gene on the X chromosome, which normally produces a protein (FMRP) essential for brain development. In FXS, this gene is either silenced or doesn’t produce enough of the protein, leading to developmental challenges.
Rationale for correct answer:
B. Arm flapping is a common behavioral feature of fragile X syndrome, often triggered by excitement, stress, or overstimulation. It reflects the syndrome’s link to autistic-like behaviors and sensory processing challenges.
Rationale for incorrect answers:
A. Strabismus may occur in many children but is not a defining or common feature of fragile X syndrome.
C. Vision deficit is not a hallmark feature of fragile X. Visual problems can occur in any child but are not specifically linked to this condition.
D. Nevus flammeus, also known as a “port-wine stain,” this is a vascular birthmark unrelated to fragile X syndrome.
Take home points
- Fragile X syndrome features include: intellectual disability, speech/language delay, autistic-like behaviors (hand flapping, poor eye contact), hyperactivity, anxiety, and sometimes aggression.
- Physical features may include long face, large protruding ears, and (post-puberty) macroorchidism.
- Behavioral manifestations (such as arm flapping) are often the most noticeable signs in childhood and affect learning and social integration.
- Early intervention with speech therapy, occupational therapy, and behavioral support is critical.
Nursing Test Bank
Naxlex Comprehensive Predictor Exams
Related Questions
Correct Answer is C
Explanation
Support from other parents of children with special health care needs is a powerful coping mechanism. Shared experiences foster understanding, reduce isolation, and help families adapt to challenges more effectively than professional advice alone at times.
Rationale for correct answer:
C. Support between parents of special children is extremely valuable. Parents often find strength, comfort, and practical advice from those who have experienced similar challenges. These peer connections promote resilience, normalize feelings, and provide ongoing emotional support.
Rationale for incorrect answers:
A. Assuming the nurse has not provided enough support is inaccurate. While professional support is important, peer support adds a unique dimension that nurses cannot replace.
B. Viewing the relationship as potentially unhealthy is incorrect. Unless it becomes exclusive or fosters dependency, peer support is beneficial, not harmful.
D. Confidentiality is not a concern here as long as families willingly share their own experiences. The nurse must only safeguard protected health information.
Take home points
- Parent-to-parent support is highly effective in managing stress and promoting coping for families of children with special needs.
- Nurses should encourage peer connections through support groups or networking opportunities.
- Professional support and peer support complement each other, ensuring holistic family care.
- Confidentiality concerns arise only if health professionals disclose private information, not when parents voluntarily share.
Correct Answer is C
Explanation
Safe and compassionate care during the transition from curative to palliative treatment in pediatric oncology emphasizes supporting both the child and family. Nurses play a key role in ensuring that families remain central to decision-making, honoring their values, preferences, and cultural beliefs.
Rationale for correct answer:
C. Families are the primary caregivers and advocates for the child. Including them ensures respect for their values, promotes understanding of the goals of care, and helps them adjust emotionally to the transition. This approach enhances shared decision-making, maintains dignity, and strengthens coping mechanisms.
Rationale for incorrect answers:
A. The health care professionals should make the decision about the child’s care excludes the family, undermines trust, and disregards their role as the child’s advocate. Ethical pediatric care requires family participation in all major decisions.
B. Continuing aggressive treatment without benefit may increase suffering, prolong distress, and is not consistent with palliative principles. Hope can be reframed toward comfort, quality of life, and meaningful moments.
C. Palliative care is available across settings, including hospitals, hospices, and home. The choice depends on family preference, resources, and the child’s needs.
Take home points
- In pediatric palliative care, family-centered decision-making is essential.
- Nurses should support the family by providing honest information, emotional support, and guidance.
- Hope is not lost, rather, it is redirected toward comfort, dignity, and quality of life.
- Palliative care can occur in hospitals, hospices, or at home depending on the child and family’s wishes.
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