6. GUIDELINES FOR MANAGEMENT
3 important steps (to be followed in order):
Assess and ensure adequate ventilation and oxygenation:
Remember, what kills these patients is asphyxia.
Do not hesitate to intubate a child with respiratory distress, even if the bleeding seems to be mild. In infants most of the blood will remain in the lung parenchyma and filling the airspaces.
If the bleeding site (or at best, lung) is identified, keep the ipsilateral side dependent so the unafected lung can sustain ventilation. Selective intubation and ventilation of the unaffected lung can also be attempted, particularly if the bleeding site is in the right lung.
If initial assessment is good, monitor oxymetry. Monitor blood gases if there is evidence of mild hypoventilation.
Ensure that the patient adequately clears secretions, this
is of particular importance in patients with CF, were secretions clearance
is already a problem. Except for patients with pulmonary trauma, there is
no contraindication for respiratory therapy in patients with hemoptysis.
This will help clear the airways from clots and avoid the loss of functional
lung units and significant V/Q mismatch.
Assess and maintain intavascular volume:
Even though death from exsaguination is rare in children, development of hypovolemia or significant anemia (which children with PH usually already have) will complicate management.
Check vital signs, capillary refill and look for orthostatic changes.
Start a good IV line (the largest bore possible), have blood typed and ready for transfusion if the need arises.
Check platelet count and obtain a coagulation profile. Ensure that the patient is not on any drugs that will impair coagulation. DDAVP (IV or intranasal, depending on the patient's condition) can be tried.
Monitor hematocrit, expect it to drop in the first few
hours if the patient has had an acute onset of massive bleeding and is evaluated
shortly after it started.
Determine the cause and site (if possible) of the bleeding:
Good history, physical exam, chest X ray and CBC to start. A chest CT (with contrast) may add valuable information in the child with an abnormal X ray, particularly when bronchiectasis or congenital malformations are suspected.
In children with underlying conditions (like CF) cause not an issue. For previously healthy children, keep in mind possible causes mentioned on previous sections.
Bronchoscopy has been advocated as indicated in any child with acute bleeding or without a clearcut cause for the bleeding. Usually possible to look for upper airway lesions or identify lung segment from which blood is coming from (rarely bleeding site will actually be identified). Through bronchoalveolar lavage adequate specimens for hemosiderin stains can be obtained.
Bronchoscopy has also a therapeutic potential. Different procedures have been described to help stop the bleed: segmental lavage with ice-cold saline, bronchial blockage with balloon catheter, local instillation of vasoactive agents, &c.
Angiography can be attempted in the patient with persistent
massive bleeding. It will not always identify bleeding vessel, but it may
identify tortous vessels that are possible sources. Embolization of these
vessels with gelfoam pieces, metal coils or thrombotic agents can be performed
at same time and is usually succesful in stopping bleeding.
Further management is dictated by the etiology of the bleeding.
If bleeding persists in spite of aggresive intervention, lobectomy should
be considered.
Click here to go to Suggested Readings