- Difficulty chewing or swallowing
- Difficulty breathing
- Difficulty walking or doing daily tasks
- Symptoms of Werdnig-Hoffmann syndrome
Werdnig-Hoffmann syndrome is a degenerative disease that has no cure and, therefore, its treatment is done to relieve symptoms and help the patient to overcome the limitations created by the syndrome, which are related to the lack of strength in muscles throughout the body.
Thus, treatment may vary according to the age of the patient, the muscles affected and the severity of the symptoms:
Difficulty chewing or swallowing
In these cases, it is usually necessary to place a nasogastric tube up to the stomach or perform a gastrostomy, which is a tube that goes directly from the skin to the stomach, to allow feeding the patient without the risk of choking or aspiration of food into the lungs, which can cause pneumonia, for example. Here's how to use the sona: How to feed a person with probe.
The difficulty in chewing or swallowing is related to the lack of strength in the muscles of the mouth and neck, which may arise in the baby or develop throughout life, as the disease progresses.
Difficulty breathing
Breathing difficulty is one of the most frequent symptoms of Werdnig-Hoffmann syndrome in babies and, therefore, it may be necessary to have a tracheostomy, which is the opening of a small hole in the throat, shortly after birth to facilitate breathing. and improve the child's quality of life.
However, in the mildest cases, where the difficulty in breathing is less severe, the treatment can be done only with the use of a CPAP, which is a device that forces the air into the lungs, replacing some of the strength of the muscles of the body. breath. Learn more about this device at: Nasal CPAP.
Difficulty walking or doing daily tasks
The lack of strength in the body's muscles always causes limitations for the patient with Werdnig-Hoffmann syndrome, preventing him from performing the simplest daily activities, such as walking, eating, getting dressed or getting dressed, for example.
Thus, physiotherapy is always indicated for all cases of the disease, not to increase muscle strength, but to help the patient to develop strategies that facilitate the performance of daily tasks, such as using a wheelchair to move around or using cutlery specials to eat.
Symptoms of Werdnig-Hoffmann syndrome
Symptoms of Werdnig-Hoffmann's syndrome may appear shortly after birth or gradually set in, appearing only after 10 years of age. In the case of arising in the baby, the disease usually causes:
- Generalized muscle weakness; Excessive joint flexibility; Difficulty in controlling the head, even after 3 months; Involuntary contraction of the muscles, including the tongue; Difficulty in feeding or swallowing.
In children or adults, the symptoms are related to muscle weakness, which usually starts in the legs or arms and spreads through the other muscles of the body.
Werdnig-Hoffmann syndrome is a genetic disease that arises when there is a family history of the disease in both the father and the mother's family. Thus, when there is a disease in the family, it is recommended to do genetic counseling to assess, through genetic tests, if there is a chance that the baby will be born with the disease.
