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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

Categories
Drugs Acting on the Immune System Nursing

Introduction to the Immune Response and Inflammation

Introduction to the Immune Response and Inflammation

Body’s Defenses

  • Barrier defenses
  • Cellular defenses
  • Inflammatory response
  • Immune response

Barrier Defenses

  • Skin
    • Protects the internal tissues and organs of the body
  • Mucous membrane
    • Lines the areas of the body that are exposed to external influences but do not have skin protection
  • Gastric acid
    • Secreted by the stomach in response to many stimuli
  • Major histocompatibility complex
    • Distinguishes between self-cells and foreign cells

Types of Cellular Defenses

  • Mononuclear phagocyte system (MPS)
    • Composed of:
      • Thymus gland
      • Lymphatic tissue
      • Leukocytes
      • Lymphocytes
      • Numerous chemical mediators

Types of White Blood Cells—Leukocytes—Produced by the Body

Types of White Blood Cells Leukocytes Produced by the Body
Types of White Blood Cells Leukocytes Produced by the Body

 

 

Types of Leukocytes

Types of Leukocytes
Types of Leukocytes

 

 

The Inflammatory Response Hageman Factor (Factor XII)

  • A chemical in the plasma activated by cell injury
  • Responsible for activating three systems in the body
    • The kinin system
    • The clotting cascade: starts blood clotting
    • The plasminogen system: starts the dissolution of blood clots

The Role of Bradykinin

  • Causes local vasodilation
  • Stimulates nerve endings to cause pain
  • Causes the release of arachidonic acid
  • This release of arachidonic acid causes the release of autocoids

Types of Autocoids Released

  • Prostaglandins
    • Some augment the inflammatory reaction and some block it
  • Leukotrienes
    • Some can cause vasodilation and increased capillary permeability and some can block the reactions
  • Thromboxanes
    • Cause local vasoconstriction and facilitate platelet aggregation and blood coagulation

Clinical Presentation

  • Calor (heat)
    • Caused by increased blood flow
  • Tumor (swelling)
    • Caused by fluid that leaks into the tissues
  • Rubor (redness)
    • Caused by the increase in blood flow due to vasodilation
  • Dolor (pain)
    • Caused by the activation of pain fibers

Inflammatory Response

Inflammatory Response
Inflammatory Response

 

 

Immune Response

  • Specific invasions stimulate specific responses through the immune system
    • Lymphocytes produced in the bone marrow can develop into T lymphocytes or B lymphocytes
    • Other identified lymphocytes include natural killer cells and lymphokine-activated killer cells
      • These cells are aggressive against neoplastic or cancer cells and promote rapid cellular death

Types of T Cells

  • Effector or cytotoxic T cells
  • Helper T cells
  • Suppressor T cells

Function of T Cells

  • Effector or cytotoxic T cells
    • Found throughout the body
    • Aggressive against non-self cells
    • Can directly destroy foreign cells or mark cell so other cells can destroy them
  • Helper T cells
    • Stimulate the activity of B cells and effector T cells
  • Suppressor T cells
    • Monitor the chemical activity in the body
    • Act to suppress B-cell and T-cell activity when the foreign antigen is under control

Cell-Mediated Response

Cell-Mediated Response
Cell-Mediated Response

 

 

The Role of the B Cell

  • Programmed to identify specific proteins or antigens
  • Involved in humoral immunity
  • Produces antibodies or immunoglobulins

Humoral Immune Response

Humoral Immune Response
Humoral Immune Response

 

 

Response to the Varicella Virus

Response to the Varicella Virus
Response to the Varicella Virus

 

 

Other Mediators in the Immune Response

  • •nterferons
    • Prevent viral replication and suppress malignant cell replication and tumor growth
  • Interleukins
    • Chemicals secreted by active leukocytes to influence other leukocytes
  • Tumor necrosis factor (TNF)
    • Chemical released by macrophages; inhibits tumor growth and can cause tumor regression

Conditions That Cause Problems Involving the Immune System

  • Neoplasm
  • Viral invasion
  • Autoimmune disease
  • Transplant rejection

Theories of Autoimmune Disease

  • Result of response to a cell that was invaded by a virus, leading to antibody production to similar cells
  • In a state of immunosuppression, the suppressor T cells do not suppress autoantibody production
  • There is a genetic predisposition to develop autoantibodies