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Medical Surgical

Huntington’s Disease

Huntington;s disease is a chronic, hereditary disease of the nervous system that results in progressive involuntary choreiform (dance-like) movements and dementia.  Researchers believe that glutamine abnormally collects in certain brain cell nuclei, causing cell death.  Huntington’s disease affects men and women of all races. It is transmitted as an autosomal dominant genetic disorder. Each child of a parent with Huntington’s has a 50% risk of inheriting the illness. Onset usually occurs between 35 and 45 years of age.

CLINICAL MANIFESTATIONS

  • The most prominent clinical features are abnormal involuntary movements (chorea), intellectual decline, ans emotional disturbance.
  • Constant writhing, twisting and uncontrollable movements of the entire body as the disease progresses.
  • Facial movements produce tics and grimaces; speech becomes slurred, hesitant, often explosive, and then eventually unintelligible.
  • Chewing and swallowing are difficult, and aspiration and choking are dangers.
  • Gait becomes disorganized, and ambulation is eventually impossible; patient is eventually confined to a wheelchair.
  • Bowel and bladder control is lost.
  • Progressive intellectual impairment occurs with eventual dementia.
  • Uncontrollable emotional changes occur but become less acute as the disease progresses. Patient may be nervous, irritable, impatient. During the early stages of illness: uncontrollable fits of anger; profound , often suicidal depression; apathy; or euphoria.
  • Hallucinations, delusions, and paranoid thinking may precede appearance of disjointed movements.
  • Patient dies in 10 to 15 years from heart failure, pneumonia, or infection or as a result of a fall or choking.

ASSESSMENT AND DIAGNOSTIC FINDINGS

  • Diagnosis is made on the basis of clinical presentation, positive family history, and exclusion of other causes.
  • Imaging studies, such as computed tomography (CT), and magnetic resonance imaging (MRI), may show atrophy of striatum.
  • A genetic marker for Huntington;s disease has been located. It offers no hope pf cure or even specific determination of onset.

MEDICAL MANAGEMENT

No treatment stops or reverse the process; palliative care is given.

  • Medications such as phenothiazines (haloperidol), butyrophenones, and thioxanthenes, which block dopamine receptors, and reserpine  and tetrabenazine. Anti-parkinsonism therapy (L-dopa) may improve chorea and temporarily decrease rigidity in some patients.
  • Motor signs are continually assessed and evaluated. Akathisia (motor restlessness) in the overmedicated patient is dangerous and should be reported.
  • Psychotherapy aimed at allaying anxiety and reducung stress may be beneficial; antidepressants are given for depression or suicidal ideation.
  • Patients needs and abilities are the focus of treatment.

NURSING MANAGEMENT

  • Reinforcing understanding that Huntington;s disease takes emotions, physical, social and financial tolls on every member of the patients family.
  • Encourage genetic counseling, long-term psychological counseling, marriage counseling, and financial and legal support.
  • Teach patient and family about medications, including signs indicating need for change in dosage and medication.
  • Address strategies to manage symptoms (chorea, swallowing problems, ambulation problems, or altered bowel or bladder function).
  • Arrange for consultation with a speech therapist, if needed.
  • emphasize the need for regular follow-up.
  • Refer for home care nursing assistance, respite care, day care centers, and eventually skilled long-term care to assist patient and family to cope.
  • Provide information about the Huntington’s Disease Foundation of America, which gives information, referrals, education, and support for research.

 

Reference:

Handbook for brunner & Suddarth’s textbook in Medical-Surgical Nursing 11th edition by Joyce Young Johnson

Lippincott Williams & Wilkins pp.450-452

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Medical Surgical

Cancer of the Cervix

              

Cancer of the cervix is predominantly (90%) squamous cell cancer and can include adenocarcinomas. It is less common than it once was because of early detection by the Pap test, but it remains the third most common reproductive cancer in women. it occurs most commonly between the ages of 35 and 45 years but can occur in women as young as 18 years. Risk factors vary from multiple sex partners to smoking to chronic cervical infection (exposure to HP virus).

 

CLINICAL MANIFESTATIONS:

  • Cervical cancer is most often asymptomatic. When discharge, irregular bleeding, or bleeding after sexual intercourse occurs, the disease may be advanced.
  • Vaginal discharge gradually increases in amount, becomes watery, and finally is dark ans foul-smelling because of necrosis of the tumor mass.
  • Bleeding occurs at irregular intervals between periods or after menopause, may be slight (enough to spot the undergarment), and is usually noted after mild trauma (intercourse, douching, or defecation). As disease continues, bleeding may persist and increase.
  • Nerve involvement, producing excruciating pain the back and legs, occurs as cancer advances and tissues outside the cervix are invaded, including the fundus and lymph glands anterior to the sacrum.
  • Extreme emaciation and anemia, often with fever due to secondary infection and abscesses in the ulcerating mass, and fistula formation may occur in the final stage.

ASSESSMENT AND DIAGNOSTIC FINDINGS

  • Pap smear and biopsy results show severe dysplasia, HGSIL, or carcinoma in situ.
  • Abnormal Pap test may be followed by biopsy, dilation and curettage (D & C), computed tomography (CT), magnetic resonance imaging (MRI), intravenous utrography (IVU), cystogram and barium radiopgraphs.

MEDICAL MANAGEMENT

Disease may be staged based on the International Classification staging system or TNM classification to determine treatment as well as progress of the cancer.

  • Conservative treatments include cryotherapy (freezing with nitrous oxide), laser therapy, loop electrosurgical excision procedure (LEEP), or conization (removing a cone shaped portion of the cervix).
  • Simle hysterectomy if invasion is less than 3 mm. Radical trachelectomy is an alternative to hysterectomy.
  • For invasive cancer , radical hysterectomy, radiation (external-beam or brachetherapy), or chemotherapy (cisplatin, carboplatin, and paclitaxel [Taxol] or a combination of these approaches may be used.
  • For recurrent cancer, pelvic exenteration is considered.
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Diabetes Insipidus

 

http://www.mirage-samoyeds.com

 

Diabetes insipidus is a disorder of the posterior lobe of the pituitary gland due to a deficiency of vasopressin, the antidiuretic hormone (ADH). it is characterized by polydipsia and polyuria. Diabetes insipidus may be (1) secondary, related to head trauma, brain tumor, or surgical ablation or irradiation of the pituitary gland or infection of the central nervous system or metastatic tumors (lung or breast); (2) nephrogenic (faliure of the renal tubules to respond to ADH), possibly related to hypokalemia, hypercalcemia, and a variety of medications (eg, lithium, demeclocycline); (3) primary (hereditary), with symptoms possibly beginning t birth (defect in pituitary gland).

The disease cannot be controlled by limiting the intake of fluids because loss of high volumes of urine continues even without fluid replacement. Attempts to restrict fluids cause the patient to experience an insatiable carving for fluid and to develop hypernatremia and severe dehydration.

Clinical Manifestations

  • Polyuria: enormous daily output of very dilute urine (specific gravity 1.001 to 1.005). Primary diabetes insipidus may have ab abrupt onset or an insidious onset in adults.
  • Polydipsia: patient experiences intense thirst, drinking 2 to 20 liters of fluid daily, with a special craving for cold water.
  • Polyuria continues even without fluid replacement.

Assessment and Diagnostic Findings

  • Fluid deprivation test: fluids are withheld for 8 to 12 hours until 3% to 5% of the body weight is lost. Inability to increase specific gravity and osmolality of the urine during test is characteristic of diabetes insipidus.
  • Urine specific gravity, serum osmolality, and serum sodium levels may be obtained.

Medical Management

Objectives of the therapy are to ensure adequate fluid replacement, to replace vasopressin, and to search for and correct the underlying intracranial pathology. Treatment for diabetes insipidus of nephrogenic origin involves using thiazide diuretics, mild salt depletion, and prostaglandin inhibitors (eg. ibuprofen, indomethacin, and aspirin).

Vasopressin Replacement

  • Desmopressin (DDAVP), administered intranasally, 1 or 2 administrations daily to control symptoms.
  • Lypressin (Diapid), absorbed through nasal mucosa into blood; duration may be short for patients with severe disease.
  • Intramuscular administration of ADH (vasopressin tannate in oil) every 24 to 96 hours to reduce urinary volume (shake vigorously or warm; administer in the evening, rotate injection sites to prevent lipodystrophy)

Fluid Conservation

  • Clofibrate, a hypolipidemic agent, has an antidiuretic effect on patients who have some residual hypothalamic vasopressin.
  • Chlorpropramide (Diabinese) and thiazide diuretics are used in mild forms to potentiate the aciton of vasopressin; may cause hypoglycemic reactions.

Nursing Management

  • Encourage and support patient undergoing studies for possible cranial lesion.
  • Instruct patient and family members about follow-up care and emergency measures.
  • Advise patient to wear a medical identification bracelet and to carry medication information about the disorder at all times.
  • Use caution with administration of vasopressin if coronary artery disease is present because of vasoconstrictive action of this drug.

 

Reference:

Joyce Young Johnson et. al Handbook for Brunner & Suddarth’s Textbook of Medical-Surgical Nursing 11th edition

Lippincott Williams & Wilkins pp.297-299

 

 

 

Categories
Medical Surgical

Addison’s Disease (Primary Adrenocortical Insufficiency)

Wikipedia

Addison’s disease is caused by a deficiency of cortical hormone. It results when the adrenal cortex function is inadequate to meet the patient’s need for cortical hormones. Autoimmune or idiopathic atrophy of the adrenal glands is responsible for 80% to 90% of cases. Other causes include surgical removal of both adrenal glands or infection (tuberculosis or histoplasmosis) of the adrenal glands. Inadequate secretion of adrenocorticotrophic  hormone (ACTH) from the primary pituitary gland results in adrenal insufficiency. Symptoms may also result from sudden cessation of exogenous adrenocortical hormone therapy, which interferes with normal feedback mechanisms.

Clinical Manifestations:

Clinical manifestations include muscle weakness, anorexia, gastrointestinal symptoms, fatigue, emaciation, dark pigmentation of the skin and mucous membranes, hypotension, low blood glucose, low serum sodium and high serum potassium. The onset usually occurs with nonspecific symptoms. Mental changes (depression, emotional lability, apathy and confusion) are present in 60% to 80% of patients. In severe cases, disturbance of sodium and potassium metabolism may be marked by depletion of sodium and water and severe, chronic dehydration.

Addisonian Crisis

This medical emergency develops as the disease progresses. Signs and symptoms include:

  • Cyanosis, fever, and classic signs of circulatory shock: pallor, apprehension, rapid and weak pulse, rapid respirations, and low blood pressure
  • Headache, nausea, abdominal pain, diarrhea, confusion and restlessness
  • Slight overexertion, exposure to cold, and acute infections, decrease salt intake and may lead to circulatory collapse, shock and death
  • Stress of surgery or dehydration from preparation for diagnostic tests or surgery may precipitate addisonian or hypotensive crisis
  • Decreased blood glucose and sodium levels, increased serum potassium (hyperkalemia), and leukocytosis.

Assessment and Diagnostic Findings

Primary adrenocortical insufficiency:

  • greatly increased plasma ACTH (>22.0 pmol/L)
  • serum cortisol low normal or lower than normal (<165 nmol/L)
  • decreased blood glucose (hypoglycemia)
  • decreased sodium levels (hyponatremia)
  • increased serum potassium (hyperkalemia)
  • increased white cell count (leukocytosis)

Medical Management

Immediate treatment is directed toward combating shock.

  • Restore blood circulation, administer fluids, monitor vital signs, and place patient in a recumbent position with legs elevated
  • Administer intravenous hydrocortisone, followed by 5% dextrose in normal saline
  • Vasopressor amines may be required if hypotension persists
  • Antibiotics may be prescribed for infection
  • Oral intake may be initiated as soon as tolerated
  • If adrenal gland does not regain function, lifelong replacement of corticosteroids and mineralocorticoids is required
  • Dietary intake should be supplemented with salt during times of gastrointestinal losses of fluids through vomiting and diarrhea

NURSING PROCESS: The Patient with Addison’s Disease

Assessment

Assessment focuses on fluid imbalance and stress

  • Check blood pressure from a lying to standing position; check pulse rate
  • Assess skin color and turgor
  • Assess history of weight changes, muscle weakness, and fatigue
  • Ask patient and family about onset of illness or increased stress that may have precipitated crisis

Diagnosis

Nursing Diagnoses

  • Deficient fluid volume related to inadequate fluid intake and fluid loss secondary to inadequate adrenal hormone secretion
  • Deficient knowledge about need for hormone replacement and dietary modification

Collaborative Problems/Potential Complications

Addisonian crisis

Planning and Goals

Goals may include improving fluid balance, improving response to activity, decreasing stress, increasing knowledge about need for hormone replacement and dietary modifications, and ensuring absence of complications.

Nursing Interventions

Restoring Fluid

  • Record weight changes daily
  • Assess skin turgor and mucous membranes
  • Instruct patient to report increased thirst
  • Monitor lying, sitting, and standing blood pressures frequently
  • Assist patient in selecting, and encouraging patient to consume, food and fluids that assist in restoring and maintaining fluid and electrolyte balance (eg, foods high in sodium during gastrointestinal disturbances and very hot weather). Include a dietitian for added guidance.
  • Assist patient and family in learning to administer hormone replacement and to modify dosage during illness and stress
  • Provide written and verbal instructions about mineralocorticoid and glucocorticoid therapy.

Improving Activity Intolerance

  • Avoid unnecessary activities and stress that might precipitate a hypotensive episode
  • Detect signs of infection or presence of stressors that may have triggered the crisis
  • Provide a quiet, nonstressful environment during acute crises; carry out all activities for patient
  • Explain all procedures to reduce fear and anxiety
  • Explain rationale for minimizing stress during acute crisis

Monitoring and Managing Complications (Addisonian Crisis)

  • Assess for signs and symptoms of crisis: circulatory collapse and shock
  • Avoid physical and psychological stress, including exposure to cold, overexertion, infection, and emotional distress
  • Initiate immediate treatment with intravenous fluid, glucose, and electrolytes, especially sodium; corticosteroid supplements; and vasopressors
  • Avoid patient exertion; anticipate and take measures to meet patient’s needs
  • Monitor symptoms, vital signs, weight, and fluid and electrolyte balance to evaluate return to precrisis state
  • Identify factors that led to crisis

 

Reference:

Joyce Young Johnson et.al Handbook for Brunner & Suddarth’s Textbook of Medical-Surgical Nursing 11th edition

Lippincott Williams & Wilkins pp; 21 – 24