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Pulmonary and Respiratory Health and FSHD
Yes. Respiratory involvement can be seen. Evaluation of the symptoms and signs of respiratory insufficiency should be sought during routine clinic visits in patients with moderate to severe FSHD. Regular monitoring of respiratory function is suggested as one might experience insufficiency over a long period of time without presenting signs. Symptomatic respiratory insufficiency can be initially managed with nighttime non-invasive pressure support e.g. a BiPAP machine. In very severe cases, patients may require the use of a ventilator. For FSHD patients with respiratory insufficiency, in standard practice, trauma (ER, ICU), surgery and anesthesiology settings, care should be taken not to suppress respiratory drive with narcotics unless it is a situation of palliative care. In trauma (ER, ICU), surgery and anesthesiology settings, it is important to notify the doctors about FSHD and any respiratory problems the patient might have or be at risk for. Oxygen supplementation can be detrimental to FSHD patients with hypercarbic (high CO2) respiratory failure and lead to worsening CO2 levels. Oxygen should generally not be administered unless BiPAP or similar ventilatory support is also being used. Your physician and a pulmonologist can help you periodically monitor CO2 levels in the office or pulmonary function lab in the hospital. Pulmonary and Respiratory Health and FSHD Fatigue is often part of FSHD because the muscles of FSHD patients have to work harder than normal muscles, but fatigue may also have additional causes. Some FSHD patients have been found to have respiratory impairment, including sleep apnea and lower than normal forced vital capacity. The causes have not yet been definitively proven, but many doctors believe that FSHD may be an important factor. If you feel fatigue, discuss it with your doctor. If while sleeping you breathe in a labored manner or momentarily stop breathing, this may indicate sleep apnea or other respiratory impairment; again, discuss it with your doctor. Because some doctors, even experienced neurologists, don’t associate FSHD with respiratory problems, your doctor may be reluctant to order respiratory tests; be persistent if you feel fatigue or sleep problems. The doctor may order breathing tests including forced vital capacity and nocturnal oxymetry tests, and blood tests such as a blood gas test. These are non-invasive tests that generally don’t require a hospital stay. A sleep study is a more complex test that may require an overnight hospital stay. If indicated by the tests, the doctor may prescribe CPAP, BI-PAP or other mechanical ventilation at night to help breathing, increase oxygen and improve sleep. CPAP, BI-PAP and similar mechanical ventilation devices are small machines that blow air into a patient’s nose, which assists the patient in exhaling. The air is delivered by a hose running from the machine to a mask around the patient’s nose or a similar apparatus. The doctor may order the tests to be repeated periodically to ensure that the settings on the machine are appropriate. A respiratory therapist (RT) is a health professional trained in respiratory health and problems. An RT should be part of the team that monitors respiratory health. The doctor and RT may recommend stacked breathing exercises. These exercises involve a facemask attached to a football-shaped plastic bladder. A helper holds the facemask around the mouth of the person doing the exercise. The person inhales rapidly several times in succession without exhaling, while the helper squeezes the bladder to push air into the person’s lungs. This exercises the chest muscles and increases oxygen intake. These exercises don’t require much time and a family member can help an FSHD patient do them. FSHD patients who can’t walk may be at risk of developing blood clots on long airplane flights. On the advice of their doctors, some people take an anti-blood clotting prescription medication before flights to reduce the risk of clotting. Links to recent articles about respiratory problems in FSHD 2010 FSH Society International Patient and Researcher Network Meeting J Neurol Sci. 2007 Dec 15;263(1-2):49-53. Epub 2007 Jun 26. Semin Respir Crit Care Med. 2002 Jun;23(3):231-8. Neurology. 2005 Jan 25;64(2):401. Neurology. 2004 Jul 13;63(1):176-8. * * * * * * * * * Health Information About Facioscapulohumeral Muscular Dystrophy (FSHD) * * * * * * * * * The information on this website is provided for general informational and educational purposes only. The FSH Society and this website do not provide medical advice or recommendations. Licensed physicians and other medical professionals who are familiar with an individual’s specific health situation should be consulted for diagnosis and treatment of Facioscapulohumeral Muscular Dystrophy and any other medical conditions. Neither the FSH Society nor any contributor to this website can be liable or responsible for any result derived from the use of this material. TEXT SIZE
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Can Respiratory Insufficiency Occur in FSHD?