Sleep and NIV (Raf) Flashcards
(14 cards)
Bipap and not tolerating it, what are the causes?
Blowing into the eyes Poor mask and headgear fit High leak Inappropriate settings Not de-sensitized with mask Poor sensing - dyssynchony Lack of humidification
Differential for central hypoventilation?
Drugs - narcotic, anticonvulsant
Infection
Metabolic - hypoglcemia, fatty acid disorder, metabolic alkalosis
Structural - CNS hemorrhage, Arnold chiari malformation type 2, myelomeningocele
Intrinsic: congenital (CCHS), late onset (ROHAD)
Sleep related breathing abnormalities in prader willi syndrome?
- Increased amount of REM sleep
- Disturabances in circadian rhythm and excessive daytime sleepiness
- Obesity and hypotonia—>OSA
- Absence of ventilatory response to peripheral chemoreceptor stimulation—>abnormal arousals during sleep
- Obesity reduces central chemoreceptor sensitivity, which is improved with growth hormone
- But there has been sudden death in PWS kids started on growth hormone
- Need to do overnight PSG before initiation of GH in child with PWS, repeat PSG in a few weeks and yearly
What is late onset central hypoventilation?
- A trigger such as: pneumonia, severe obesity or cor pulmonale cause hypoventilation
- ROHAD - rapid onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation
Gene for CCHS?
PHOX2B (autosomal dominant)
Diagnostic criteria for CCHS?
- There has to be evidence of hypoventilation with no other obvious cause for hypoventilation: no neuromuscular disease, cardiac disease, pulmonary disease
- Hypoventilation (paCO2>60) - in particular during quiet sleep
- onset in first year of life
- Hypercapneic ventilatory challenge—it sounds like this is part fo the diagnostic criteria (not sure if this is separate from PSG)
- Investigations while waiting for genetic results:
- Metabolics - inborn error
- MRI brain
- Echo
- CXR +/- CT
Monitoring for complications of CCHS:
- Cardiac: heart block, prolonged sinus pauases, pulmonary hypertension–>annual 72 hour Holter and echo
- Hirschpsrung disease–>clinical monitoring. If concerning symptoms, then contrast enema and rectal suction biopsy
- Cognitive deficits–>Annual neurocognitive assessment
- Tumors of neural crest origin:
- NPARM: abdo imaging and urine catecholamines q3 months for first 2 years, then q6 months till age 7 years, (then every year)
- PARM with 20/28-20/33: annual chest and abdominal imaging
- PSG: every 6 months till age 3 years, then every year
- All patients: annual echo, Holter, neurocognitive assessment
Supplies for emergency tracheostomy kit?
- Obturator of current tube
- Two extra tracheostomy tubes - current size and size below
- Fully charged suction machine, suction catheters
- Normal saline syringes for installation
- Prepared trach ties
- emergency alogirthm
- phone number
- bagger with mask and connector if child is ventilated
Contraindications/relative contraindications for NIV
- Recent facial or upper airway surgery or burn
- Poor glottic function and inability to protect the upper airway
- Patient preference
- CCHS, requiring ventilation during infancy - since they would require ventilation for significant portion of day and developmental concerns
- copious respiratory secretions
Neuromuscular patient with viral illness who comes into hospital. Baseline saturation of 93%.
Need to implement cough augmentation techniques like cough assist if saturation <95% on room air (either with or without NIV). Cough assist has been shown to decrease chance of intubation.
Lung volume recruitment maneuvers for patient with NMD?
- Stacked breaths (no exhalation in between inspiration) via:
- Lung volume recruitment bag
- volume cycled NIV
- Glossopharyngal breathing
Methods for cough augmentation in NMD?
Inspiration:
* Stacked breaths (no exhalation in between inspiration) via:
* Lung volume recruitment bag
* volume cycled NIV
* Glossopharyngal breathing
Expiration:
* Manual chest wall or upper abdominal thrust
* Mechanical exsufflation with negative pressure
Which method of cough augmentation is most effective?
Mechanical insufflation/exsufflation
Respiratory considerations in a patient with neuromuscular disease?
- Ineffective cough clearance–>airway clearance (eg. cough assist)
- Swallowing dysfunction–>tube feeding, sialorrhea
- Sleep disordered breathing during REM sleep–>non-REM SDB and hypoventilation so use of NIV
Later progression to diurnal hypercapea->daytime ventilation (?invasive ventilation)
(See alogirthm for more details)
Describe components of cough reflex arc
Receptor:
- mechanical receptors from pharynx to terminal bronchiole, diaphragm, pleura. Chemical receptors - eg. capsaicin
Affarnet: vagus, glossopharyngeal
Cough centre: medulla pons
Effarent: vagus, phrenic, spinal motor nerve to diaphragm, abdominal wall muscles