Stephen E. Kirkby, MD^1,2 and Alpa V. Patel, MD¹
Division of Pediatric Pulmonary and Sleep Medicine; Department of Pediatrics¹
Division of Pulmonary, Critical Care and Sleep Medicine; Department of Internal Medicine²
Nationwide Children’s Hospital and Ohio State University Columbus, Ohio

Pulmonary rehabilitation (PR) is  an important therapy for patients with chronic and advanced lung diseases, providing benefits of improved physical conditioning and optimized psychological health.  There is a clear role for adult patients with COPD.¹ Furthermore, PR has been shown to have positive effects in adults with interstitial lung disease (ILD) and pulmonary hypertension (PH).^2-3 While there is a paucity of evidence of formal PR in cystic fibrosis (CF), exercise programs in general provide benefit in CF and non-CF bronchiectasis.⁴

Important components of successful PR have recently been described.  Key features include an  initial (pre-PR) assessment of physical fitness, respiratory symptoms, and nutritional status;  structured exercise training that is individually prescribed and monitored to best achieve improvements, and close clinical follow-up.   Participation in PR also provides important opportunities to optimize behavioral and psychological aspects of a patient’s care, improve adherence, and promote education.¹  Therefore, PR programs are commonly utilized for patients with advanced lung disease undergoing evaluation for lung transplantation.  Lung transplant candidates benefit from improving exercise capacity, endurance, muscle strength and overall physical functioning as they prepare for transplant.⁵ A transplant team can also use the PR program to ensure proper patient adherence and commitment to therapy, while providing important education. Following lung transplant, PR is often an essential component of recovery. ⁶

The role of formal PR programs in pediatric patients is poorly understood.  While adult PR programs are common in many communities, formal PR centers for children are few and referrals may be underutilized.   Adolescents and older children may be able to participate in PR structured in a similar fashion to adult programs, but younger children are unlikely to be able to do the same types of exercises and interventions.  Effective pediatric PR must be individually tailored to a child’s age, functional capacity, and psychological ability to engage.  Our group and others have demonstrated that a structured pulmonary rehabilitation program has positive effects on children with asthma, particularly those with co-morbid obesity.^7-8  Home based PH may also be utilized in this asthmatic population.⁹

While there is very limited published data on the effectiveness of PR in children with advanced lung disease, we believe that there is benefit in those patients old enough and physically able to participate.   We have utilized PR in children with advanced CF lung disease, ILD and PH, including those awaiting lung transplantation.  While our experience has not been published, we believe that a pediatric-focused exercise program has improved physical function and endurance.  It has also seemed to have benefit on the nutritional and psychological well-being of these chronically ill children.   Our pediatric transplant center has utilized PR regularly in children old enough to participate in the pre-transplant period, as well as following surgery.   Choi, et al have recently published a case report of PR being a successful intervention in a 10 year-old following transplant for bronchiolitis obliterans.¹⁰  We advocate for more children’s hospitals to develop formal PR programs, and for further clinical investigation into the benefits it may provide.

References

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2. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014 Oct 6;(10):CD006322. doi: 10.1002/14651858.CD006322.pub3. PMID: 25284270.
3. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M; ESC Scientific Document Group. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1;37(1):67-119. doi: 10.1093/eurheartj/ehv317. Epub 2015 Aug 29. PMID: 26320113.
4. Burtin C, Hebestreit H. Rehabilitation in patients with chronic respiratory disease other than chronic obstructive pulmonary disease: exercise and physical activity interventions in cystic fibrosis and non-cystic fibrosis bronchiectasis. Respiration. 2015;89(3):181-9. doi: 10.1159/000375170. Epub 2015 Feb 12. PMID: 25676797.
5. Langer D. Rehabilitation in Patients before and after Lung Transplantation. Respiration. 2015;89(5):353-62. doi: 10.1159/000430451. Epub 2015 Apr 30. PMID: 25924770.D
6. Pehlivan E, Balcı A, Kılıç L, Kadakal F. Preoperative Pulmonary Rehabilitation for Lung Transplant: Effects on Pulmonary Function, Exercise Capacity, and Quality of Life; First Results in Turkey. Exp Clin Transplant. 2018 Aug;16(4):455-460. doi: 10.6002/ect.2017.0042. Epub 2017 Sep 30. PMID: 28969527.
7. Kirkby S, Rossetti A, Hayes D Jr, Allen E, Sheikh S, Kopp B, Patel A. Benefits of pulmonary rehabilitation in pediatric asthma. Pediatr Pulmonol. 2018 Aug;53(8):1014-1017. doi: 10.1002/ppul.24041. Epub 2018 May 7. PMID: 29736958.
8. Basaran S, Guler-Uysal F, Ergen N, Seydaoglu G, Bingol-Karakoc G, UfukAltintas D. Effects of physical exercise on quality of life, exercise capacity and pulmonary function in children with asthma. J Rehabil Med. 2006; 38: 130– 135.
9. Bingöl Karakoç G, Yilmaz M, Sur S, Ufuk Altintas D, Sarpel T, Güneter Kendirli S. The effects of daily pulmonary rehabilitation program at home on childhood asthma. Allergol Immunopathol (Madr). 2000 Jan-Feb;28(1):12-4. PMID: 10757852.
10. Choi EJ, Kim W, Jeon JY, Ko EJ, Yu J, Choi SH, Lee SH, Sung IY. Intensive pulmonary rehabilitation in a pediatric lung transplantation patient: A case report. Medicine (Baltimore). 2021 Apr 30;100(17):e25523. doi: 10.1097/MD.0000000000025523. PMID: 33907101; PMCID: PMC8084064.