Sunday, February 7, 2010

Sick Growing Child: Duchenne’s Muscular Dystrophy

 What is Muscular Dystrophy?

It is a group of inherited disorders that lead to progressive degeneration of skeletal muscles, apparently from lack of a protein (merosin) that is necessary for muscle contraction


 What causes it?

The cause of MD is unknown, but there may be a genetic influence for most of the major types. Some forms of MD are transmitted as autosomal dominant or recessive traits, whereas others are sex-linked.


 What is its pathophysiology?

The exact pathophysiology mechanisms are unknown, but three theories have been advocated.

a. Vascular theory
This theory suggests that a lack of blood flow causes the typical degeneration of muscle tissue.

b. Neurogenic theory
This theory proposes a disturbance in nerve-muscle interaction.

c. Membrane theory
This theory that cell membranes are genetically altered, causing a compromise in cell integrity.



 What is its pathogenesis?

Duchenne muscular dystrophy is caused by a mutation of the dystrophin gene at locus Xp21.


 What are the types of MD?

a.Congenital Myotonic Dystrophy

Inherited as.. Autosomal dominant trait
Predominant Symptom Severe myotonia
Diagnosis Serum analysis and muscle biopsy


b. Fascioscapulohumeral Muscular Dystrophy

Inherited as.. Dominant trait carried on chromosome 4
Predominant Symptom Facial weakness
Diagnosis Serum analysis and muscle biopsy

c. Pseudohyopertrophic Muscular Dystrophy (Duchenne’s Disease)

Inherited as.. Sex-linked recessive trait
Predominant Symptom
Diagnosis Muscle biopsy, electromyography and serum creatine phosphokinase
analysis



 What are the clinical signs and symptoms of Duchenne’s MD?

- have a history of meeting motor milestones
- waddling gait and difficulty climbing stairs
- walk on their toes
- positive for Gower’s sign
- speech and swallowing becomes difficult
- difficult to lift a young child
- calf muscles are hypertrophied
- muscle weakness
- scolosis of the spine and fractures of long bones
- wheel chair dependent
- tachycardia
- pneumonia



 What are the ways to manage this disease?

a. Nursing
- Encourage the patient to remain ambulatory for as long as possible
- Help plan the child and family plan a program of active and passive daily ROM
- Apply splints and braces
- Encourage a low-calorie, high protein diet
- Encourage a high intake of fiber and fluids
- Advocate for a stool softener if necessary
- Provide support for parents

b. Medical
- Administer prednisone (dosage of 0.75 mg/kg or less)

c. Surgical
- Myoblast transfer therapy



I.

Nursing Diagnosis:
Impaired physical mobility related to progressive muscle wasting

Plan:
The client will remain physically active.

Nursing interventions:
Reinforce physical therapy regimen.

Encourage activity (e.g., school attendance, swimming)

Encourage the use of adaptive equipment as needed (e.g., back brace)


II.

Nursing Diagnosis:
Self care deficit related to progressive muscle weakness


Plan:
The client will be able to perform activities of daily living(ADL) as long as possible

Nursing interventions:
Instruct the family in ways to adapt the home as needed ( grab bars, overhead sling, raised toilet, wheelchair access)

Focus on the things the child can do to prevent frustration.

Maintain the child’s independence as long as possible (e.g., via a n electric wheelchair or portable phone)



------------------------------------------------------
Sources:

Adele Pillitteri, 5th Ed. Maternal & Child Health Nursing: Care of the Childbearing & Childrearing Family. Volume 2 page 1635

Klossner, Hatfield, Introductory Maternity & Pediatric Nursing p.724

Ignatavicus, Workman. Medical – Surgical Nursing Critical Thinking for Collaborative Care. p. 1183

http://books.google.com.ph/books?id=-WP4y6Ve3eMC&pg=PA1462&lpg=PA1462&dq=nursing+diagnosis+for+duchennes+muscular+dystrophy&source=bl&ots=F7Z3nDO1Aa&sig=6hBQrCtoUwxTN2Sfw2FFFyFlpUs&hl=tl&ei=C6FuS-CRKY2A7QOVzNH6BQ&sa=X&oi=book_result&ct=result&resnum=5&ved=0CCQQ6AEwBA#v=onepage&q=&f=false

http://www.nlm.nih.gov/medlineplus/ency/article/000705.htm

http://www.ncbi.nlm.nih.gov/pubmed/2012511

http://en.wikipedia.org/wiki/Duchenne_muscular_dystrophy

Friday, February 5, 2010

HIGH RISK INFANT: Cystic Fibrosis (CF)

I. Definition

- is a genetic disease that affects many organs and lethally impairs pulmonary function
- When first described, CF was called “fibrocystic disease of the pancreas” but additional research reveals that this disorder represents a major dysfunction of all exocrine glands
- is hereditary and transmitted as an autosomal recessive trait


II. Etiology

- abnormality of the long arm of chromosome 7
- caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator(CFTR)


III. Incidence/ Prevalence

- US: 1 in 3,300 white children and 1 in 16, 300 black children
- Males and females are affected equally

IV. Pathophysiology

The normal gene produces a protein, CFTR, which serves as a channel through which chloride enters and leaves cells. The mutated gene blocks chloride movement, which brings upon the apparent signs of CF.

The blocking of chloride transport results in a change in sodium transport; thus in turn results in abnormal secretions of the exocrine (mucous producing) glands that produce thick, tenacious, rather than thin, free-flowing secretion normally produced.

This abnormal mucus leads to obstruction of the secretory ducts of the pancreas, liver, and reproductive glands. Thick mucus obstructs the respiratory passages, causing trapped air and over inflation of the lungs. In addition, the sweat and salivary glands excrete excessive electrolytes, specifically sodium and chloride.



V. Types

Type Error
I Zero CFTR is produced

II CFTR is not processed correctly, so no protein gets to the cell membrane

III The CFTR chloride pathway is regulated differently than normal, but transfer of CFTR occurs

IV The chloride current is not conducted properly, so transfer of CFTR is slowed

V Synthesis of CFTR is abnormal; however, CFTR is transferred


VI. Clinical Manifestations

Pancreas Involvement

• Steatorrhea
• Protuberant abdomen
• malnutrition – emanciated extremities and loose, flabby folds of skin on their buttocks
• Fat-soluble vitamin deficiencies
= CELIAC SYNDROME

Others:

 Meconium ileus
 Rectal prolapse



Lung Involvement

• Pooled secretions of the bronchioles
• Secondary emphysema
• Bronchiectasis
• Pneumonia
• Atelectasis
• Anterior-posterior diameter of the chest becomes enlarged
• Respiratory acidosis


Sweat Gland Involvement
- Newborn tastes salty when kissed



VII. Diagnostic exam

• Sweat Chloride Analysis

The defect in chloride movement prevents absorption of sodium chloride in the sweat glands; thus more chloride than normal is present in the sweat.

The sweat chloride test is positive for CF when the chloride level in the sweat ranges between 60 and 200 mEq/L (mmol/L), compared with the normal value of 20 mEq/L.

• Duodenal analysis
• Serum immunoreactive trypsin
• Pulmonary testing
• Stool analysis






----------------
Sources:

Adele Pillitteri, 5th Ed. Maternal & Child Health Nursing: Care of the Childbearing & Childrearing Family. Volume 2 pages 1269-1274

Klossner, Hatfield, Introductory Maternity & Pediatric Nursing p.829 – 837

Ignatavicus, Workman. Medical – Surgical Nursing Critical Thinking for Collaborative Care. p. 607 - 609

http://en.wikipedia.org/wiki/Cystic_fibrosis#Pathophysiology

http://ccn.aacnjournals.org/cgi/reprint/25/4/46.pdf

POSTPARTAL COMPLICATION: Laceration

I. Definition
- is an open wound wherein the edges are often jagged and tissues are torn apart




II. Etiology
- with difficult/ precipitate births
- in primigravidas
- with the birth of a large infant (more than 9 lb)
- with the use of a lithotomy position
- episiotomy
- use of forceps/ vacuum extraction for delivery



III. Types

A. Cervical Lacerations
- usually found on the sides of the cervix, near the branches of the uterine artery
- occurs immediately after the delivery of the placenta

B. Vaginal Lacerations
- rare
- easier to assess

C. Perineal Lacerations
- occur when a woman is placed in a lithotomy position


Four Categories

*First degree - vaginal mucous membrane & skin of the perineum to the fourchette
*Second degree - vagina, perineal skin, fascia, levator ani muscle & perineal body
*Third degree - entire perineum & reaches the external sphincter of the rectum
*Fourth degree - entire perineum & some of the mucous membrane of the rectum



IV. Signs/Symptoms
- fundus is firm on palpation yet bleeding is heavy, bright red, and continues in a steady trickle or flow
- pain may be constant
- bruising may be apparent






----------------
Sources:

Kozier, Erbs., 8th Ed. Fundamentals of Nursing. Volume 2 p.904

Adele Pillitteri, 5th Ed. Maternal & Child Health Nursing: Care of the Childbearing & Childrearing Family. Volume 1 pages 595, 605, 614, 661- 662

Zabriskie, 10th Ed. Obstetrics for Nurses. p.278

Klossner, Hatfield, Introductory Maternity & Pediatric Nursing by Klossner & Hatfield p.456, 459

Uterine Inversion

I. Definition

Uterine inversion is a condition in which the inner surface of the fundus comes out through the cervix. In other words, the uterus “turned inside out.” The placenta appears at the introitus with a mass attached.





II. Causes/ Risk Factors
- Almost always a consequence of strong traction on an umbilical cord attached to placenta implanted on the fundus
- Abnormal implantation such as placenta accrete, increta and percreta
- Manual removal of the placenta
- Uterine atony
- Congenital or myometrial weakness
- Previous uterine inversion





III. Types
A. Complete
- the uterus extends beyond the level of the cervix with a dark, beef-looking mass inside the vagina or outside the introitus

B. Incomplete
- the uterus does not extend beyond the cervix, with a mass only palpable or visible at the cervix.





IV. Classification

A. Acute
- occur immediately after, or moments after, delivery; cervix is not yet constricted

B. Sub-acute
- occur immediately after, or moments after, delivery; cervix is constricted

C. Chronic
- inversion that occurred longer than 4 weeks postpartum




V. S/S

Uterine inversion often results in profuse bleeding which, without prompt treatment, leads to shock and even death. The severity of shock has been observed to be disproportionate to the blood loss due to underestimation of bleeding or possibly to neurogenic shock caused by immense pain. Furthermore, other complications aside from those mentioned include infection and endomyometritis, and injury to neighboring structures such as the intestines and bladder. The woman may be bradycardic because of increased vagal tone.

Placenta Previa

I. Definition
- Is low implantation of the placenta



II. Cause(s)
- Uterine abnormality
- Increased parity
- Advanced maternal age
- Past cesarean births
- Past uterine curettage
- Multiple gestation



III. Types
- It occurs in four degrees:
A. implantation in the lower rather than in the upper portion of the uterus (low-lying placenta)
B. marginal implantation (the placenta edge approaches that of the cervical os)
C. implantation that occludes a portion of the cervical os (partial placenta previa)
D. implantation that totally obstructs the cervical os (total placenta previa).




IV. Signs and Symptoms
- Painless bleeding, bright red in color, at beginning of cervical dilation

Monday, January 25, 2010

Plan of Visit # 1 (example)

Plan of Visit #1


Name of patient: Date of Visit:
Age: Case:
Address:



General objectives:

After __ days of student nurse-family interaction, the family will be able to acquire adequate knowledge, proper skills and positive attitude towards promotion of health & prevention of illness.



Specific Actions:

After 45 minutes of student nurse-family interaction, the family will be able to:

1. express cooperation with the student nurse
2. explain the purpose & importance of home visits
3. identify health related problem
4. cite health promotion activities being practiced
5. set contract and agree on the next schedule of the home visit



Nursing Actions:

1. establish rapport with the family members
1.1 wear proper uniform and name plate for identification
1.2greet the family
1.3 introduce oneself

2. discuss to the family the purpose of the visit
2.1 to assess the living condition of the client
2.2 to give health teachings regarding the prevention and control of diseases
2.3 to give nursing care to the sick

3. assess each member of the family by utilizing the following:
3.1 IDB
3.2 FCI
3.3 FNCP

4. inquire from the family regarding their health practices

5. inform the family of the next home visit