16 December, 2013

A rough couple of weeks

Last week while Robert was home waiting out his low counts, Ben came home with strep.  By the time we caught it, both he and Allie were infectious little germ machines. Kasie segregated them to different ends of the house, washed hands, walls, doorknobs, husband, children and anything else that would hold still somehow managing to keep him healthy through the contagious period.

 Robert spent this week in the hospital in the attempt to resolve his lung issues.  They performed another bronchoscopy and ran a battery of every test they could think of.  They haven't completely confirmed it yet, but the Pulmonologist is strongly leaning towards a diagnosis of a condition called Plastic Bronchitis.  Here is a paste from the interweb;

"Plastic bronchitis is a rare condition characterized by the formation and expectoration of long, branching casts of the bronchial tubes. Primarily a pediatric problem, plastic bronchitis is usually associated with some type of underlying pulmonary disease including bronchiectasis, cystic fibrosis, bronchial asthma, and allergic bronchopulmonary aspergillosis. Symptoms include violent coughing, wheezing, and episodes of severe dyspnea.
Pathologic examination demonstrates that the bronchial casts diagnostic of plastic bronchitis are composed of layers of mucus thickened by evaporation or absorption of fluid and the breakdown products of inflammatory cells.
During bronchoscopic examination, the tracheobronchial tree frequently is found to be filled with white, cheesy material too soft to grasp and remove with forceps but too thick to suction. During episodes of severe distress, patients have been known to cough out a complete cast of the tracheobronchial tree.

  • In plastic bronchitis, inability to clear mucus casts from the lungs results in pulmonary complications including impaired gas exchange, chronic or persistent infection, atelectasis, and permanent tissue damage.
  • Mucus hypersecretion, which occurs as a response to inflammation secondary to infection and epithelial tissue damage, further exacerbates the incidence of mucus plugging and cast formation.
  • Retained secretions provide a culture medium for bacterial pathogens. Consequent infections produce still more mucus, initiating a vicious cycle of mucus obstruction, recurrent bouts of pneumonia, bacterial colonization, and, finally, respiratory failure.
In addition to therapeutic interventions to treat underlying pulmonary disease, patients with plastic bronchitis require aggressive bronchial hygiene, including daily Airway Clearance Therapy."

From what I understand the condition rare, but when it is diagnosed it is more frequently associated with congenital heart conditions.  We were told that there was no known correlation to the cancer treatment, and the doctor could only find one other case with an ALL Leukemia patient.

Basically what it means for Robert is that he has a substance in his lungs that he can't clear, and his body has been making him cough incessantly trying.  New meds, breathing treatments, and a new therapy involving a vest that aggressively agitates his chest are part of the new routine. 

 So hopefully we're on the right track to getting him feeling better.  

Today is the 3rd anniversary of the diagnosis. Chemo is scheduled to end in 2014, things are going to be a bit different next year.