The Ups and Downs of Treating Respiratory Disease in Our Orangutan Mias

Part 2 • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • •

By: Cindy

Cossaboon

Intermittent Symptoms as far back as 1997

Nasal discharge

Dermatitis around throat sac

Cough

Congestion

Diarrhea

Lethargy

Health History • • • • • • • • • • • • • • • • • • • • • • • • • •

Dr. Jennifer Taylor-Cousar

Assistant Professor, Departments of Internal Medicine and Pediatrics, Pulmonary Division, Associate Adult CF Program Director

Leslie Schamaun, RRT

Lead Respiratory Therapist

National Jewish Hospital • • • • • • • • • • • • • • • • • • • • • • • • • •

CF Diagnostic Criteria Mias’ signs and symptoms

Clinical Features

Chronic Sinopulmonary Disease

Gastrointestinal abnormalities

1.Chronic nasal drainage and chronic sinusitis

(see CT scan, Fig 1.)

2. Chronic cough and bronchiectasis

3. Bronchoscopic cultures demonstrating S.aureus,

P. aeruginosa

Fecal elastase 20 ug/g, improvement in chronic flatus

and abdominal distension with initiation of pancreatic

enzyme replacement therapy

Evidence of CFTR dysfunction Sweat test QNS (quantity not sufficient)

CFTR genetics + for CFTR mutations

Ciliary biopsy Normal

National Jewish Research • • • • • • • • • • • • • • • • • • • • • • • • • •

Chart created Dr. Jennifer Taylor-Cousar

Antibiotics every other month

Pneumococcal vaccine

Done October 24, 2012

Nasal Scrapings with electron Microscope

Chest and Sinus CT Scan

Stool sample for pancreatic function

DNA analysis for Mias, Nias and Hesty

Steps for Treatment • • • • • • • • • • • • • • • • • • • • • • • • • •

Albuterol

Saline

Tobramycin/Colistimethate

Drug Treatment • • • • • • • • • • • • • • • • • • • • • • • • • •

In CF, primary ciliary dyskinesia causes chronic lung disease

Mias’ ciliated cells are normal in structure making it less likely the cause of chronic lung disease.

Nasal Scrapings with electron Microscope • • • • • • • • • • • • • • • • • • • • • • • • • •

Chest and Sinus CT Scan • • • • • • • • • • • • • • • • • • • • • • • • • •

Results: Inflammation of the tissues in his sinuses. Widespread disease in his airways that is more evident in the lower part of his lungs (and worse on the right). The different segments of the air sac are thick

Chest and Sinus CT Scan • • • • • • • • • • • • • • • • • • • • • • • • • •

Humans with have CF values over 200ug/g

Age is a factor in humans

Need more samples

Did start Mias on pancrelipase with great results

Stool sample for pancreatic function • • • • • • • • • • • • • • • • • • • • • • • • • •

Mias: 20 ug/g Robin: 45 ug/g Nias: 47 ug/g Sally: 23 ug/g Hesty: 83 ug/g

National Jewish Research • • • • • • • • • • • • • • • • • • • • • • • • • •

DNA analysis of Mias’ DNA Using primers for human CFTR (Cystic Fibrosis Transmembrane Regulator gene) Designing some specific primers Mias had two disease causing mutations Looking for Mias’ relatives for comparison Using blood from Nias and Hesty for comparison

Nias’ results came back with zero mutations John’s Hopkins lab is still working on extracting Hesty’s DNA from feces.

Calling all Mias Relatives!! • • • • • • • • • • • • • • • • • • • • • • • • • •

Any known health information about these individual Any respiratory issues, gastrointestinal issues, or no issues at all

Studbook ID Birth Date Sex Status Location Name

2119 6 Mar 1987 Male Living Perth Dinar

2512 18 Aug 1992 Female Living Toronto Sekali

1455 30 Aug 1975 Female Living Fort Worth Chantek

2359 4 Jun 1990 Male Died Toronto Kartiko

2024 4 Oct 1985 Female Living Toronto Ramai/Rimb

2418 1 Aug 1991 Female Died Birminghm Kira

THANK YOU!! • • • • • • • • • • • • • • • • • • • • • • • • • •