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Diseases » Adams Nance syndrome » Treatments
 

Treatments for Adams Nance syndrome

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Book Excerpts: Treatment of Adams Nance syndrome

Treatments of Adams Nance syndrome: Online Medical Books

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Seizures/Convulsions: Treatment
(In a Page: Signs and Symptoms)

  • Generalized status epilepticus (continuous or recurrent seizure activity without a return to baseline for >30 minutes) is a medical emergency and should be treated aggressively with IV antiepileptic medications
  • Remove offending intoxicants or medications
  • Correct metabolic abnormalities as necessary
  • Numerous anticonvulsant medications are available for acute and chronic use
    –Select the most appropriate agent on the basis of the clinical situation, seizure type, and side effect profile
    –Combination anticonvulsant therapy may be effective for patients refractory to a single agent
  • Vagus nerve stimulators are effective in patients refractory to anticonvulsant therapy
  • Epilepsy surgery also may be effective in controlling seizures in carefully selected surgical candidates who are refractory to chronic anticonvulsant medication
  • Lifestyle modification and avoidance of triggers (e.g., sleep deprivation, alcohol)
  • » READ BOOK EXCERPT ONLINE »

    Source: In a Page: Signs and Symptoms, 2004

    Seizures – Childhood: Treatment
    (In A Page: Pediatric Signs and Symptoms)

    • In the acute setting: First evaluate and secure airway, breathing, and circulation
      –IV benzodiazepines (lorazepam) is first line of treatment; in the absence of IV access, rectal diazepam may be used
      –IV fosphenytoin is the second line of treatment and provides longer seizure suppression
      –If not sufficient, phenobarbital can be added
      –Constant evaluation of the airway during treatment, and, if necessary, intubation is critically important
    • If the seizure continues despite these medications, anesthesia with pentobarbital, midazolam, or propofol may be used to suppress the refractory seizures
    • Long-term prophylaxis can be obtained by one or a combination of anti-epileptic medications
      –Focal seizures: Carbamazepine, oxcarbazepine, gabapentin, levetiracetam, or valproate
      –Absence seizures: Ethosuximide or valproate

    » READ BOOK EXCERPT ONLINE »

    Source: In A Page: Pediatric Signs and Symptoms, 2007

    Seizures – Neonatal: Treatment
    (In A Page: Pediatric Signs and Symptoms)

      • Evaluate and secure airway, breathing, and circulation
        –Benzodiazepines or phenobarbital infused intravenously can stop the seizures
    • If there is reason to suspect hypoglycemia, even if blood glucose level cannot be quickly established, treat with glucose intravenously
    • Treat hypocalcemia with calcium gluconate
    • Treat hypomagnesemia with magnesium sulfate
    • Meningitis and HSV encephalitis require intravenous antibiotics and acyclovir, respectively
    • Depending on the etiology, the infant may stay on phenobarbital for varying duration of time
    • Glucose transporter deficiency can be treated with ketogenic diet
    • For pyridoxine deficiency, pyridoxine 50–100 mg injected intravenously during an EEG recording can be both diagnostic and therapeutic

    » READ BOOK EXCERPT ONLINE »

    Source: In A Page: Pediatric Signs and Symptoms, 2007

    Seizures, generalized tonic-clonic: Emergency interventions
    (Handbook of Signs & Symptoms (Third Edition))

    If you witness the beginning of the seizure, first check the patient’s airway, breathing, and circulation, and ensure that the cause isn’t asystole or a blocked airway. Stay with the patient and ensure a patent airway. Focus your care on observing the seizure and protecting the patient. Place a towel under his head to prevent injury, loosen his clothing, and move any sharp or hard objects out of his way. Never try to restrain the patient or force a hard object into his mouth; you might chip his teeth or fracture his jaw. Only at the start of the ictal phase can you safely insert a soft object into his mouth.

    If possible, turn the patient to one side during the seizure to allow secretions to drain and to prevent aspiration. Otherwise, do this at the end of the clonic phase when respirations return. (If they fail to return, check for airway obstruction and suction the patient if necessary. Cardiopulmonary resuscitation, intubation, and mechanical ventilation may be needed.)

    Protect the patient after the seizure by providing a safe area in which he can rest. As he awakens, reassure and reorient him. Check his vital signs and neurologic status. Make sure to carefully record these data and your observations during the seizure.

    If the seizure lasts longer than 4 minutes or if a second seizure occurs before full recovery from the first, suspect status epilepticus. Establish an airway, start an I.V. line, give supplemental oxygen, and begin cardiac monitoring. Draw blood for appropriate studies. Turn the patient on his side, with his head in a semi-dependent position, to drain secretions and prevent aspiration. Periodically turn him to the opposite side, check his arterial blood gas levels for hypoxemia, and administer oxygen by mask, increasing the flow rate if necessary. Administer diazepam or lorazepam by slow I.V. push, repeated two or three times at 10- to 20-minute intervals, to stop the seizures. If the patient isn’t known to have epilepsy, an I.V. bolus of dextrose 50% (50 ml) with thiamine (100 mg) may be ordered. Dextrose may stop the seizures if the patient has hypoglycemia. If his thiamine level is low, also give thiamine to guard against further damage.

    If the patient is intubated, expect to insert a nasogastric (NG) tube to prevent vomiting and aspiration. Be aware that if the patient hasn’t been intubated, the NG tube itself can trigger the gag reflex and cause vomiting. Make sure to record your observations and the intervals between seizures.

    » READ BOOK EXCERPT ONLINE »

    Source: Handbook of Signs & Symptoms (Third Edition), 2006

    Seizures, absence: Patient counseling
    (Professional Guide to Signs & Symptoms (Fifth Edition))

    Explain the purpose of any diagnostic tests, such as computed tomography scans, magnetic resonance imaging, and EEGs. Teach the patient and his family about these seizures and how to recognize their onset, pattern, and duration. Include the child’s teacher and school nurse in the teaching process, if possible. If the seizures are being controlled with drug therapy, emphasize the importance of strict compliance.

    » READ BOOK EXCERPT ONLINE »

    Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

    Seizures, generalized tonic-clonic: Emergency interventions
    (Professional Guide to Signs & Symptoms (Fifth Edition))

    If you witness the beginning of the seizure, first check the patient’s airway, breathing, and circulation, and ensure that the cause isn’t asystole or a blocked airway. Stay with the patient and ensure a patent airway. Focus your care on observing the seizure and protecting the patient. Place a towel under his head to prevent injury, loosen his clothing, and move any sharp or hard objects out of his way. Never try to restrain the patient or force a hard object into his mouth; you might chip his teeth or fracture his jaw. Only at the start of the ictal phase can you safely insert a soft object into his mouth.

    If possible, turn the patient to one side during the seizure to allow secretions to drain and to prevent aspiration. Otherwise, do this at the end of the clonic phase when respirations return. (If they fail to return, check for airway obstruction and suction the patient if necessary. Cardiopulmonary resuscitation, intubation, and mechanical ventilation may be needed.)

    Protect the patient after the seizure by providing a safe area in which he can rest. As he awakens, reassure and reorient him. Check his vital signs and neurologic status. Be sure to carefully record these data and your observations during the seizure.

    If the seizure lasts longer than 4 minutes or if a second seizure occurs before full recovery from the first, suspect status epilepticus. Establish an airway, start an I.V. line, give supplemental oxygen, and begin cardiac monitoring. Draw blood for appropriate studies. Turn the patient on his side, with his head in a semi-dependent position, to drain secretions and prevent aspiration. Periodically turn him to the opposite side, check his arterial blood gas levels for hypoxemia, and administer oxygen by mask, increasing the flow rate if necessary. Administer diazepam or lorazepam by slow I.V. push, repeated two or three times at 10- to 20-minute intervals, to stop the seizures. If the patient isn’t known to have epilepsy, an I.V. bolus of dextrose 50% (50 ml) with thiamine (100 mg) may be ordered. Dextrose may stop the seizures if the patient has hypoglycemia. If his thiamine level is low, also give thiamine to guard against further damage.

    If the patient is intubated, expect to insert a nasogastric (NG) tube to prevent vomiting and aspiration. Be aware that if the patient hasn’t been intubated, the NG tube itself can trigger the gag reflex and cause vomiting. Be sure to record your observations and the intervals between seizures.

    » READ BOOK EXCERPT ONLINE »

    Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

    Seizures, simple partial: Patient counseling
    (Professional Guide to Signs & Symptoms (Fifth Edition))

    After the seizure, instruct the patient to record his seizures. Also, emphasize the importance of complying with the prescribed drug regimen and maintaining a safe environment.

    » READ BOOK EXCERPT ONLINE »

    Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

    Seizures, generalized tonic-clonic: Nursing considerations
    (Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series)

    Closely monitor the patient for recurring seizure activity. Prepare him for a computed tomography scan or magnetic resonance imaging and EEG. Monitor therapeutic drug levels. Provide a safe environment and institute seizure precautions. Continue to monitor the patient’s vital signs and respiratory status. Provide supplemental oxygen, as indicated.

    Patient teaching

    Advise the patient’s family to observe and record his seizure activity to ensure proper treatment. Emphasize the importance of strict compliance with the drug regimen and warn the patient about its possible adverse effects. Stress the importance of regular follow-up appointments for blood studies. Provide information on alcohol or drug cessation programs if the seizure was related to withdrawal or abuse.

    » READ BOOK EXCERPT ONLINE »

    Source: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series, 2007

    Seizures, complex partial: Patient counseling
    (Signs & Symptoms: A 2-in-1 Reference for Nurses)

    Offer emotional support to the patient and his family. Teach them how to cope with seizures. Discuss safety measures to take during a seizure.

    » READ BOOK EXCERPT ONLINE »

    Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007

    Seizures, generalized tonic-clonic: Patient counseling
    (Signs & Symptoms: A 2-in-1 Reference for Nurses)

    Advise the patient’s family to observe and record his seizure activity to ensure proper treatment. Emphasize the importance of strict compliance with the drug regimen, and warn the patient about adverse reactions. Also, stress the importance of regular follow-up appointments for blood studies.

    » READ BOOK EXCERPT ONLINE »

    Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007

    Seizures, simple partial: Patient counseling
    (Signs & Symptoms: A 2-in-1 Reference for Nurses)

    After the seizure, instruct the patient to record his seizures. Also, emphasize the importance of complying with the prescribed drug regimen and maintaining a safe environment.

    » READ BOOK EXCERPT ONLINE »

    Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007

    Seizures, absence: Nursing considerations
    (Nursing: Interpreting Signs and Symptoms)

    ▪ Administer an anticonvulsant, as ordered.

    ▪ Assess neurologic status, noting episodes of possible absence seizures.

    Patient teaching

    ▪ Teach the patient and family about the condition and its treatments.

    ▪ Explain signs and symptoms that require immediate attention.

    ▪ Emphasize the importance of follow-up medical care.

    ▪ Discuss the need to wear medical identification.

    » READ BOOK EXCERPT ONLINE »

    Source: Nursing: Interpreting Signs and Symptoms, 2007

    Seizures, complex partial: Nursing considerations
    (Nursing: Interpreting Signs and Symptoms)

    ▪ After the seizure, remain with the patient to reorient him to his surroundings and to protect him from injury.

    ▪ Keep the patient in bed until he's fully alert, and remove harmful objects from the area.

    ▪ Prepare the patient for diagnostic tests, such as EEG, computed tomography scan, or magnetic resonance imaging.

    Patient teaching

    ▪ Explain the disorder and its treatment.

    ▪ Offer emotional support to the patient and his family, and teach them how to cope with seizures.

    ▪ Discuss with the patient and his family safety measures to take during a seizure.

    ▪ Emphasize compliance with drug therapy.

    ▪ Stress the importance of carrying medical identification.

    » READ BOOK EXCERPT ONLINE »

    Source: Nursing: Interpreting Signs and Symptoms, 2007

    Seizures, generalized tonic-clonic: Nursing considerations
    (Nursing: Interpreting Signs and Symptoms)

    ▪ Institute seizure precautions.

    ▪ Closely monitor the patient after the seizure for recurring seizure activity.

    ▪ Prepare him for a computed tomography scan or magnetic resonance imaging and EEG.

    ▪ Administer anticonvulsants, as ordered, and monitor drug levels.

    Patient teaching

    ▪ Explain the disorder and treatment plan.

    ▪ Teach the family how to observe and record seizure activity.

    ▪ Emphasize the importance of compliance with drug therapy and follow-up appointments.

    ▪ Tell the patient to carry medical identification.

    » READ BOOK EXCERPT ONLINE »

    Source: Nursing: Interpreting Signs and Symptoms, 2007

    Seizures, simple partial: Nursing considerations
    (Nursing: Interpreting Signs and Symptoms)

    ▪ Institute seizure precautions.

    ▪ Stay with the patient during seizure activity and reassure him.

    ▪ Monitor neurologic status.

    ▪ Prepare the patient for such diagnostic tests as a computed tomography scan and EEG.

    Patient teaching

    ▪ Explain the disorder and treatment plan.

    ▪ Explain that no emergency care is necessary during a focal seizure, unless it progresses to a generalized seizure. (See “Seizures, generalized tonic-clonic,” page 552.)

    ▪ Teach the family how to record seizures.

    ▪ Tell the patient to carry medical identification.

    » READ BOOK EXCERPT ONLINE »

    Source: Nursing: Interpreting Signs and Symptoms, 2007



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