Maximum Voluntary Ventilation can help predict lung issues in Myotonic Dystrophy patients

A new study of myotonic dystrophy patients indicates that an older style test of maximum voluntary ventilation may help Myotonic Dystrophy patients know their lung function better. 

The maximal voluntary ventilation (MVV) is another measure of the neuromuscular and respiratory systems. The MVV is the total volume of air exhaled during 12 sec of rapid, deep breathing, which can be compared with a predicted MVV defined as the forced expiratory volume in 1 sec (FEV1× 35 or 40. A significant difference between the predicted and measured MVV may indicate insufficient neuromuscular reserve, abnormal respiratory mechanics, or an inadequate effort. Progressive reduction of tidal volumes during the test is consistent with neuromuscular abnormalities but also occurs with gas trapping as a result of disorders that cause airflow limitation.

Clinical_implication_of_maximal_voluntary.-ventilation-in-Myotonic-Dystrophy

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Care recommendations for patients with myotonic dystrophy

Published in 2018 is a consensus based approach for the myotonic dystrophy patient community. This gives general guidelines on how to approach, test and intervene in patients lives to achieve the most optimum outcomes.

Care-recommendations-for-adulats-with-Myotonic-Dystrophy

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Lung Function and Myotonic Dystrophy

A consortium of doctors met and came up with some definitive pathways for patients with myotonic dystrophy

A Multi-country conference was held with doctor from most major European Countries and the USA to review respiratory or lung function in patients with Myotonic Dystrophy. Patients with DM have a lot of issues with lung function and respiration. A framework or step by step checklist was reviewed and recommended at this conference. The conclusion of this conference is presented below. This is a very complex subject so print this and bring it with you to your doctor or  pulmonologist. When you have myotonic dystrophy its important to have your lung function checked every 6 months or so.

Respiratory-insufficiency-in-Myotonic-Dystrophy

Management of respiratory insufficiency is challenging in
DM because of the pathophysiology of the disease, which
affects both muscles and central respiratory pathways and
patients’ cognitive and behavioral characteristics. Although
there are no natural history data on the effects of respiratory
care on survival and on morbidity in patients with DM, results
of ventilation on survival and on better care in other
neuromuscular diseases indicate that adequate ventilatory care
may improve survival and QoL of patients with DM1 [41–43].
NIV use varies greatly among the different centers, e.g. varying
from 20 to 60% in adults with DM1, 20–40% of patients with
the congenital form, and 15–20% of patients with DM2. All
participants agreed on the need for standard assessments and
recommendations for standard of care.
Discussion between the specialists from different countries
led to the construction of initial standard protocols which are
the necessary preliminary steps for validation processes to
follow. Specifically, the workshop led to the creation of: 1) a
respiratory symptom check-list to be applied in everyday DM
clinic (Table 1); 2) a preliminary version of a screening
respiratory protocol to be applied on first assessment during
clinic (Figure 1); 3) proposal of criteria for NIV prescription to
be used specifically in patients with DM, based on the existing
ACI (Consensus Statement from the Agency for Clinical
Innovation Respiratory Network on Domiciliary Non-Invasive
Ventilation in Adult Patients) guidelines for NIV, including
recommendations for timing and tests to be performed on
follow-up assessments; 4) proposal of a secretion management
protocol (Figure 2).
The need for validation of these recommendations and for
further research to extend the evidence-base in certain key areas
was also highlighted and outline proposals to resolve these
deficiencies put forward. All participants agreed on the urge for
more natural history data and for specific pathways for
emergency care of acute respiratory insufficiency in DM.

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Watch your Weight!!! – It may affect your breathing!

 

A recently published article has great information about weight and breathing. Simple conclusion: is that being overweight with Myotonic Dystrophy can affect your breathing and respiratory function. Since respiratory failure and pneumonia are big issue with Myotonic Dystrophy pay special attention to your weight!!! It also showed that a great majority of people with DM have an abnormal body composition. ITs important to keep the weight off but you also must see a nutritionist to insure that you are getting proper nutrition and to look at your body weight/mass/BMI. Here is the summary

InDM1 patients, overweight is an independent factor for predicting TLC, and contributes independently of FIV1. Because overweight isr elated to increased work of breathing and inspiratory muscle strength is reduced inDM1, the fatigue threshold will be reached sooner. Therefore, muscle fatigue and the onset of respiratory failure will develop at an earlier stage in overweight patients, especially during increased ventilator demand. Moreover, over half of DM1patients are overweight, and nearly all patients have an abnormal body composition. To develop interventional strategies for weight loss, it will be important to categorize the individual type of body composition. Hence, preventing the development of overweight inDM1 patients may result in delaying respiratory failure and mortality in DM1.

Click below on the link for the full study

Overweight Myotonic Dystrophy

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Report of using BiPAP with Infants Newborns with Congential Form of Myotonic Dystrophy

Successful use of BiPAP in infants with congenital myotonic dystrophy.

Source

Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of Medicine, University of Hong Kong Department of Paediatrics and Adolescent Medicine, Duchess of Kent Children’s Hospital and Queen Mary Hospital, LKS Faculty of Medicine, University of Hong Kong, Hong Kong.

Abstract

Reported herein are two cases of severe phenotype of congenital myotonic dystrophy (CDM) with presentation of respiratory insufficiency at birth. The infants were successfully managed with bi-level positive airway pressure (BiPAP) via nasal mask. The use of BiPAP in infants with CDM has not been reported before. The rationale for using BiPAP is discussed. BiPAP may be more effective than continuous positive airway pressure in managing respiratory insufficiency, especially in infants with the more severe phenotype of CDM.

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