The Science Journal of the American Association for Respiratory Care

2007 OPEN FORUM Abstracts


CASE STUDY: PULMONARY REHABILITATION IN A PATIENT WITH LAM/TUBEROUS SCLEROSIS COMPLEX.

S. Farrell1, G. A. Connors1, T. Malinowski2, J. Lamberti2


INTRODUCTION:
Lymphangioleiomyomatosis (LAM) is a life-threatening lung disease affecting exclusively young women. Histologically, LAM is characterized by the diffuse, bilateral proliferation of abnormal smooth muscle cells and cystic degeneration of lung parenchyma. LAM can manifest with severe decompensation and often requires lung transplantation. Patients must retain strength and endurance to allow quality of life and especially if lung transplantation is required. We present a case study that demonstrates the effectiveness of a pulmonary rehabilitation (PR) program in helping to maintain endurance and function while awaiting transplantation.

CASE SUMMARY: Subject is a 36 year old female diagnosed with LAM/Tuberous Sclerosis Complex in January 2004. PFT data: FEV1 0.6 l, 21% predicted, DLCO 3.4 mL/mmHg/m, 16% predicted. She participated in a comprehensive 8 week, twice/week, PR program August through October 2004. Baseline PR data: 6MWT distance 1,121 ft., highest BORG 5, lowest O2 Sat 83% on 1.5 L/m O2, Hospital Anxiety and Depression Scales (HADS) scores 9 for anxiety and 6 for depression, St. George Respiratory Questionnaire (SGRQ): Total Score 57.3. Post PR data: 6MWT distance 1,140 ft., highest BORG 3, lowest O2 Sat 90% on 1.5 L/m O2, HADS score 7 for anxiety and 1 for depression. SGRQ: Total score 47.7. Twelve months later her medical condition worsened, she was hospitalized for a tension pneumothorax, evaluated and placed on the lung transplant list. Her post hospitalization 6MWT: 684 ft, highest BORG 4, lowest O2 Sat 90% on 2 L/m O2. Her condition continued to deteriorate and she was monitored and interval exercised in in maintenance PR. A maintenance exercise 6 MWT 9/2006 was: 912 ft., highest BORG 3, lowest O2 Sat 95% on 5 L/m nasal cannula. She received a single lung transplant late September 2006. Her post-lung transplant 6MWT was 1,174 ft., highest BORG 3, O2 sat 97% on RA. Her most recent 6MWT & HAD shows significant improvement.

DISCUSSION: During her participation in a comprehensive PR program her 6MWT distance remained stable with improvement in BORG.  Quality of life markers improved as HAD and SGRQ scores decreased. She maintained strength despite declining pulmonary status to be listed for lung transplantation.

Conclusion: Pulmonary rehabilitation enabled this patient to maintain adequate strength, endurance & improved her quality of life. PR also prepared the patient for lung transplantation.

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CASE STUDY: PULMONARY REHABILITATION IN A PATIENT WITH LAM/TUBEROUS SCLEROSIS COMPLEX.