Multiple Sclerosis (MS)

Definition:

Multiple Sclerosis (MS) involves an immune – mediated process in which an abnormal

response of the body’s immune system is directed against the central nervous system

(CNS).

Multiple Sclerosis (MS) is potentially disabling disease of the brain and spinal cord

(central nervous system). In MS, the immune system attacks the protective sheath that

covers nerve fibers and causes communication problems between brain and the rest of the

body. Eventually, the disease can cause permanent damage or deterioration of nerves.

Clinical Manifestation:

 Numbness or weakness in one or more limbs that typically occurs on one side of

the body at a time, or legs and trunk

 Electric – shock sensations that occur with certain neck movements, especially

bending the neck forward (Lhermitte sign)

 Tremor, lack of coordination or unsteady gait

 Slurred speech

 Fatigue

 Dizziness

 Tingling or pain in parts of the body

 Problems with sexual, bowel, and bladder function

Vision Problems;

 Partial or complete loss of vision, usually in one eye at a time, often with pain

during eye movement

 Prolonged double vision

 Blurry vision

Medical Management:

Diagnosis:

 Cerebrospinal fluid (CSF) analysis reveals elevated protein level,

increased white blood cells

 Electrophoresis of CSF shows increased myelin basic protein and

IgG bands

 Electroencephalography

 Evoked potential studies

 Computed tomography scan

 Magnetic Resonance Imaging

Treatment:

 Prednisone

 Methylprednisone

 Dexamethasone

 Chlordiazepoxide hydrochloride

 Baclofen

 Dantrolene
  Behanechol

 Oxybutynin

 Muscle stretching for spastic muscles and selective strengthening

exercises for weakness are prescribed.

 Consult with a physical therapist if the patient needs to learn how

to use assistive devices or needs to learn exercises to maintain

muscle tone and joint mobility

 A social service agency may be required to help the family deal

with the often expensive and long – term financial effect of the

disease.

Nursing Management:

1. Instruct the client to increase fluid intake and eat a balanced diet,

including low-fat, high-fiber foods and foods high in potassium.

2. Instruct the client in safety measures related to sensory loss, such as

regulating the temperature of bath water and avoiding heating pads.

3. Instruct the client in safety measures related to motor loss, such as

avoiding the use of scatter rugs and using assistive devices.

4. Provide bed rest during exacerbation.

5. Protect the client from injury by providing safety measures.

6. Place an eye patch on the eye for diplopia.

7. Monitor for potential complications such as urinary tract infections,

calculuses, decubitus ulcers, respiratory tract infections, and contractures.

8. Promote regular elimination by bladder and bowel training.

9. Instruct the client to balance moderate activity with rest periods.

10. Instruct the client to avoid fatigue, stress, infection, overheating, and

chilling.
Sepsis

Definition:

Sepsis a potentially life – threatening condition caused by the body’s response to an

infection. The body normally releases chemicals into the bloodstream to fight an

infection. Sepsis occurs when the body’s response to these chemicals is out of balance,

triggering changes that can damage multiple organ systems.

Clinical Manifestation:

 Hyperthermia

 Hypothermia

 Tachypnea

 Tachycardia

 Abdominal skin

 Warm skin, possibly rash

 Generalized Weakness

 High WBC count

 Low WBC count

 Coagulation Imbalance
Medical Management:

Diagnosis:

 Complete blood Count

 Platelet count

 White blood count

 Urinalysis

 X-ray

 Computerized tomography scan

 Ultrasound

 Magnetic resonance imaging (MRI)

Treatment:

 Cefuroxime

 Ceftin

 Ceftazidime

 Intravenous fluids

 Vasopressors

 Supportive care that includes oxygen

 Dialysis if the kidney is affected

 Surgery may be needed to remove sources of infection, such as

collections of pus (abscesses), infected tissues or gangrene.

Nursing Management

1. Monitor client’s Pulse rate, Temperature, and Respiratory rate

2. Adjust environmental factors as indicated. Remove excess clothing as necessary

3. Observe for excessively dry skin and mucous membranes.

4. Encourage a balanced diet

5. Encourage frequent position changes

6. Perform tepid sponge bath as necessary

7. Monitor for peripheral edema in the legs, back and scrotum

8. Administer antipyretics as ordered

9. Administer IV fluid as ordered by physician

10. Administer antibiotic as order by physician

Shwachman – Diamond Syndrome

Definition:

Shwachman – Diamond Syndrome is an inherited condition that affects many parts of the

body, particularly the bone marrow, pancreas, and skeletal system. Most cases of

Shwachman – Diamond syndrome are caused by mutations in the SBDS gene. In this

case, there are no SBDS mutation is found; cause of this disorder is unknown. This

condition is inherited in an autosomal recessive manner.

Clinical Manifestation:

 Short stature

 Anemia

 Bone marrow dysfunction

 Recurrent infections

 Exocrine pancreatic insufficiency

 Metaphyseal dysostosis

 Recurrent skin

 Respiratory infections

 Growth Retardation

 Hepatic Dysfunction

 Low levels of vitamins (A, D, E, and K)

Medical Management:

Diagnosis:

 Genetic Testing

Treatment:

 Pancreatic enzymes (for digestion pancreatic enzyme supplements

are included diet

 Growth factor therapy

 Antibiotics

 Surgery (may be used for skeletal abnormalities)

 Vitamin supplementation

 Blood/platelet transfusion

 Granulocyte – colony stimulating factor (G –CSF)

 Hematopoietic stem cell transplantation

Nursing Management:

1. Instruct client’s parents to administer fat – soluble vitamins and multivitamins to

the child.

2. Educate the family about the disease of their child

3. Encourage the family to consult physician for any complications of the child.

4. Monitor child’s behavior and growth

5. Monitor for any signs of infection

Hyperbilirubinemia

Definition:

Hyperbilirubinemia is an excess of bilirubin in the blood, occurring as a result of liver or

biliary tract dysfunction or with excessive destruction of red blood cells. It is classified as

conjugated or unconjugated, according to the type of bilirubin present. Jaundice is

manifested when excess bilirubin is deposited in the skin and mucous membranes

Clinical Manifestation:

 Jaundice

 Dark urine

 Ascites

 Hemorrhagic tendency

 Palmar erythema

 Gynecomastia

 Pale stools

 Fatigue

 Abdominal pain

 Weight loss

 Vomiting

Medical Management:
Diagnosis:

 Complete blood count

 Bilirubin test

 Serum aminotransferases

 Serologic screen for hepatitis

 Alkaline phosphatase

 Fractionated bilirubin

 Blood alcohol or acetaminophen levels

 Antimitochondrial antibody

 Antinuclear antibodies

 Abdominal ultrasonography

 Abdominal CT scan

 Abdominal MRI

 Endoscopic retrograde cholangiopancreatography

Treatment:

 Phototherapy

 Intravenous Immunoglobulin

 Exchange transfusion

 Feeding with breastmilk

Nursing Management:

1. Assess color of skin, sclera of eye and mucous membrane of mouth and nose for

every 8 hours.

2. Check for any sign of complication and notify physician

3. Check neurological status hourly to identify complication of bilirubin

encephalopathy

4. Check vital signs every 4 hour

5. Monitor intake and output and check urine and stool color

6. Administer medication as order

7. Provide healthy diet consult with dietician

8. Encourage patient to take plenty of fluid at least 6 – 8 glass daily if breast milk

encourage mother to breastfeed every 2 – 3 hours.

9. Check weight daily to evaluate weight loss or gain

10. Administer IV fluid if diarrhea is present

Hirschsprung Disease

Definition:

Hirschsprung disease is a birth defect in which nerve cells are missing at the end of a

child’s bowel. Normally, the bowel contains many nerve cells along its length that

control how the bowel works. When the bowel is missing nerve cells, it does not work

well. This damage causes blockages in the bowel because the stool does not move

through the bowel normally.

Clinical Manifestation:

 Incomplete intestinal obstruction

 Length of the aganglionic segment is variable

 Failure to pass meconium within 24 hours

 Constipation during first month of life

 Bile – stained emesis

 Abdominal distention

 Distended abdomen

 Reluctance to eat

 Failure to thrive

 Stool with ribbon – like appearance

Medical Management:

Diagnosis:

 Digital examination of rectum reveals absence of stool followed by

explosive release of gas

 Barium enema

 Rectal biopsy

Treatment:

Surgery

 Pull – through surgery (Laparoscopic)

 Ostomy Surgery ( Ileostomy or Colostomy)

 Decompression

 Antibiotic (Broad – spectrum antibiotics)

Nursing Management:

1. Monitor vital sign every 2 hours

2. Promote skin integrity when performing routine colostomy care.

3. Promote comfort, observe for signs of pain

4. Provide adequate nutrition in accordance with the recommended diet

5. Monitor intake and output of the client

6. Monitor signs of turgor adequate body fluids

7. Observe any signs of increased nausea and vomiting, anticipated deficit of body

fluids immediately

8. Explain about the disease process, diet, treatments, and medicines to the client’s

family.

9. Explain all the procedures that will be implemented and the benefits for client.

10. Provide oral and nasal care, gently clean the nares to relieve any irritation from

the NG tube.