Nursing Care of a Child with MOPD II and Moyamoya according to Orem’s Self-care Deficiency Theory: A Case Report

Abstract

Microcephalic osteodysplastic primitive dwarfism (MOPDII) is the most familiar form of microcephalic primordial dwarfism (MPD). The classic features of the MPD group are marked microcephaly and severe antenatal and postnatal growth retardation. Such patients also have Moyamoya disease, which includes recurrent stroke attacks and possible neurological complications. Today, it is possible to provide care for such patients with many theories. Caring for children with growth-developmental retardation with Orem's Self-care Deficiency Theory may enable them to become self-aware and increase their quality of life. The significance of nursing care for this rare disease is undeniable. In this study, the nursing care of a patient diagnosed with MOPD2 and Moyamoya was carried out based on the Self-care Deficit Nursing Theory. "Partly remedial nursing care" was provided as the patient can self-care at a moderate level, and also "supportive and educational nursing care" was provided to support the mother and other family members who provide care to the patient.

Introduction

Microcephalic osteodysplastic primitive dwarfism (MOPD II) is the most common and best-described form of microcephalic primordial dwarfism (MPC). The classic features of the MPC group are marked microcephaly and severe antenatal and postnatal growth retardation (1-4).

Children with MOPD II are dependent on others throughout their lives due to growth and development delays, and this negatively affects their ability to self-care and their quality of life. In this study, due to its rarity in Turkey, the case of MOPD II was addressed. Nursing care was planned using Orem's Self-care Deficiency Theory since children with this diagnosis cannot self-care and always need a caregiver. This study aimed to provide better care to children diagnosed with MOPD II and Moyamoya, to present an example of a nursing care plan based on Orem's Self-care Deficit Theory so that they can cope with the disease, and thus to guide and provide consultancy to the nurses.

Case Report

Key Situational Factors

1. Pregnancy and birth history

S.A. is born at 35 weeks+6 days with normal birth. She was 35 cm tall (<3rd percentile) and weighed 920 g (<3rd percentile). S.A. remained in the incubator for 36 days. She was discharged at 1150 g.

2. Disease history

The baby's weight gain and height growth were very slow until the sixth month. After the sixth month, she was diagnosed with MOPD II and Moyamoya, and the treatment process was started (Carbamazepine 3x1, Levothyroxine sodium 1x1, Acetylsalicylic acid 1x1). At 18 months, S.A. had a seizure, developed partial paralysis in her left hand and foot, and recovered after two weeks of physical therapy. She started attending special education when she was 22 months old. She has been continuing her education for eight years.

Current patient information:

Age: 13

Gender: girl

Diagnosis: MOPD II, Moyamoya

Weight: 24.5 kg (<3p) Height: 81cm (<3p) Head Circumference: 43 (microcephalic appearance)

Socio-cultural characteristics: SA has a nuclear family and has two siblings. Siblings of SA have no known disease.

Lifestyle: She can walk but gets tired quickly due to motor retardation. Although she can do many things herself, she gets support from her family because she feels exhausted. She goes from one place to another on her mother's lap or with a baby carriage. She does not like to eat, especially because she has problems chewing and swallowing food.

Interaction with the environment: Social isolation has increased during the pandemic period. He continues private education at home. There is physical and motor skill retardation.

Presence and adequacy of resources: She has social security. Their economic status is moderate.

4. Self-care

Since the child cannot fully perform self-care activities independently, she is partially dependent on the family. However, the child's self-care agent is primarily the mother.

5. Self-care strength

The level of meeting the child's self-care needs is low. Needs help in meeting self-care needs.

Self-confidence and respect: The child established eye contact while speaking. She is extrovert and sociable.

Ability to control and initiate her energy: She can perform activities of daily living at a moderate level. She has difficulty breathing when she does long exercises and walks.

Comprehension competence: Perceptual and cognitive status is moderate. The child generally understands what is being said and can do what is asked. She has difficulty making sense of some newly heard concepts. She can become irritable and stubborn when she does not understand. She is easily distracted.

Motivation: The child can express her wishes and problems verbally. When she is stubborn and cannot make sense of some things, the mother tries to motivate her by repeating or explaining the same information over and over again.

Making decisions about self-care: The child can perform basic self-care skills with help, and the mother usually makes the decisions about the child, except for the choice of clothes to wear.

Perceptual, cognitive status and communication skills: The child gives the desired answers to the questions. She plays cooperative games with the other children. She has trouble understanding new concepts. She fulfills her responsibilities in a game properly. She communicates well with her family as well as her environment.

6. Therapeutic self-care needs

Therapeutic self-care needs are examined under three sub-dimensions.

I. Universal self-care needs

Air: She has difficulty breathing because her lungs are not fully developed. The respiratory distress of the child increases, especially with excessive movement or exercise.

Nutrition: She can eat by herself. She mostly enjoys consuming foods that she can swallow without chewing (e.g., soup, pudding and yogurt).

Oral health: Her teeth are sparse and small.

Activity: The child can walk short but has difficulty climbing stairs. She can have her bath with help. Her physical activity is moderate.

Sleep: Total sleep time at night varies between 7-8 hours. She has difficulty falling asleep.

Cognitive perception: The child's level of perception and reaction to events is medium.

II-Developmental self-care needs: The child has a physical and motor developmental delay. He does not like crowds. The pandemic has increased the social isolation of children and families.

III- Health deviation self-care needs:

Difficulty in breathing

Disruption in sleep pattern

Vision problem

Hearing problem

Nutrition less than body requirement

Disproportionate growth

Oral motor problems

Social isolation

7. Lack of self-care

The power of the dependent care agent: Communication between child and mother is better than other family members. The mother is knowledgeable about her child's illness and needs.

Self-care deficit of the dependent care agent: Due to the mother's other responsibilities, she has difficulty allocating enough time to SA.

8. Nursing Process

In our study was determined that the child was deficient in self-care skills. Thus, "Partly Remedial Nursing Care" was given to the child and "Supportive and Educational Nursing Care" was given to his family (5-14). Informed onsent was obtained to conduct this case study.

.. .

.. .

.. .

Discussion

MOPD II and Moyamoya require a multidisciplinary approach, especially for nurses with significant responsibilities. Although many studies are related to treating children diagnosed with MOPD II, to our knowledge, no studies have been found addressing nursing theories and care (5-8). Providing care using nursing theories is crucial in providing a comprehensive, systematic approach and a holistic nursing perspective at every stage of care (9-14). It ensures that patients can be cared for in an evidence-based and professional manner (5-9).

In the case under consideration, it was observed that the child was able to perform activities of daily living at a moderate level, was inadequate even in basic self-care skills, such as getting dressed, and having a bath, and had nutritional deficiencies and sleep problems. For the child to perform self-care activities independently, responsibilities appropriate to her physical capacity were given in cooperation with his family. Due to the training provided to the child and the family members, the child could put on and take off his clothes and tie shoelaces. S.A., who had difficulty falling asleep, did as she was told, yet there was no change in her sleep duration. The social isolation of the child, who has been away from the rehabilitation center for a long time due to the pandemic, continues. Thanks to the planning made with the family, it was observed that other family members reduced the mother's responsibilities and burden, and the mother created time for her self-care. When the case was evaluated in general, it was observed that the child started to meet her self-care needs. It was observed that the quality of life of both the caregivers and the child has increased. Orem’s Self-care Deficiency Theory provides a general framework directly related to nursing functions and guides nurses. It can be recommended to have a more extended training period to evaluate the behavior and development of the child more comprehensively by using Orem's theory.

Cite this article as: Askin Ceran M, Bektas M. Nursing Care of a Child with MOPD II and Moyamoya according to Orem’s Self-care Deficiency Theory: A Case Report. Pediatr Acad Case Rep. 2023;2(1):6-11.

The parents’ of this patient consent was obtained for this study.

The authors declared no conflicts of interest with respect to authorship and/or publication of the article.

The authors received no financial support for the research and/or publication of this article.

References

1. Kılıç E, Utine E, Unal S, Haliloğlu G, Oğuz KK, Cetin M, et al. Medical management of moyamoya disease and recurrent stroke inan infant with Majewskiosteo dysplastic primordiald warfism type II (MOPD II). Eur J Pediatr 2012; 171:1567–71.
2. Huang-Doran I, Bicknell LS, Finucane FM, Rocha N, Porter KM, Tung YC, et al. Genetic Defects in Human Pericentrin Are Associated with Severe Insulin Resistance and Diabetes 2011; 60: 925–35.
3. Duker AL, Kinderman D, Jordan C, Niiler T, Baker-Smith CM, Thompson L, et al. Microcephalic osteodysplastic primordial dwarfism type II is associated with global vascular disease. Orphanet J Rare Dis 2021; 16: 231.
4. Perry LD, Robertson F, Ganesan V. Screening for cerebrovascular disease in microcephalic osteodysplastic primordial dwarfism type II (MOPD II): an evidence-based proposal. Pediatric Neurology 2013; 48 (4): 294-8.
5. Hall JG, Flora C, Scott CI Jr, Pauli RM, Tanaka KI. Majewski osteodysplastic primordialdwarfism type II (MOPDII): natural history and clinical finding. Am J Med Genet 2004; 130A: 55-72.
6. Galasso C, Lo-Castro A, Lalli C, Cerminara C, Curatolo P. Microcephalic osteodysplastic primordial dwarfism types I and III. Pediatr Neurol 2008; 38: 435–8.
7. Fawcett J. Contemporary Nursing Knowledge Analysis and Evaluation of Nursing Models and Theories, 2nd ed. Philadelphia: F. A. Davis Company; 2005.
8. Varcarolis EM. Caring for patients for psycho-biological disorders. In: Varcarolis EM. Essentials of Psychiatric Mental Health Nursing. A Communication Approach to Evidence-Based Care, 3rd ed. St. Louis, MO; Elsevier Saunders; 2016. p.120–30
9. Tar E. Orem Öz-Bakım Teorisine Göre Otizm Spektrum Bozukluğu Olan Çocuk ve Ailesini Tanılama Süreci: Olgu Sunumu. Güncel Hemşirelik Araştırmaları Dergisi 2021; 1(3): 126-34.
10. Başoğul C. ve Buldukoğlu K. Psikiyatrik Bakımda Orem’in “Öz Bakım Eksikliği Kuramı”nın kullanımı: Olgu Sunumu. ACU Sağlık Bil. Derg 2020; 11(4): 729-37.
11. Ahmed, H. M., Mohammed, R. E., Ibrahim, S. R. Effect of Orem Self-Care Model on school-age children and teenagers with autism and their parents. Benha University 2019 https://bu.edu.eg/staff/samahalrefaai13- publications/40019
12. Rodrigues PMS, Albuquerque MCS, Brêda MZ, Bittencourt IGS, Melo GB, Leite AA. Self-care of a child with autism spectrum by means of Social Storiesa. Escola Anna Nery 2007; 21.
13. Oliver CJ. Triage of the Autistic Spectrum Child Utilizing the Congruence of Case Management Concepts and Orem’s Nursing Theories. Professional Case Management 2003; 8(2): 66-82.
14. Yıldız FT, Kaşıkçı M. Koroner Arter Hastalığı Tanısı ile İzlenen Bireyin Orem’in Öz Bakım Yetersizlik Kuramı’na Göre Bakımı (Olgu Sunumu). Balıkesir Sağlık Bilimleri Dergisi, 2018; 7(3): 114-120.