There is no specific treatment for progressive muscular dystrophy so far, undoubtedly supportive therapy is the mainstay. Patients should be encouraged to engage in daily activities as much as possible to avoid prolonged bed rest, as inactivity can often lead to exacerbation and disability; increase nutrition, avoid over-ageing and prevent infection. Physical therapy and orthopedic treatment can prevent and improve deformity and contracture, and are important to maintain active function.
1. There is no definite program for drug treatment.
(1) Glucocorticoid: The effect on delaying the development of the disease and prolonging life has been affirmed. Prednisone 0.75mg/(kg.d) orally can improve muscle strength, side effects include weight gain, Cushing’s syndrome-like manifestations and hirsutism; the long-term efficacy of prednisone on this disease is uncertain.
(2) anti-free radical drugs to improve metabolism: vitamin C, vitamin E and coenzyme Q10, etc.;
(3) Allopurinol: treatment of Duchenne type can improve the clinical symptoms to varying degrees, CK levels have decreased; younger patients have better efficacy, regular checks of white blood cells during treatment, such as below 3000 × 106/L should be discontinued;
(4) Adenosine triphosphate, inosine, nandrolone phenylpropionate and traditional Chinese medicine can be tried;
(5) Treatment methods under research: gene therapy, stem cell transplantation, adult cell transplantation, new drug exploration.
2. It is important to take preventive measures against PMD, mainly including detection of carriers and prenatal diagnosis.
(1) Carriers can be detected through genealogical analysis: female relatives of DMD patients may be carriers, divided into.
① sure carriers: one or more male affected children’s mothers, while the patient’s aunts and uncles also have the disease;
②Likely carriers: two or more mothers of patients, but no prior evidence in maternal relatives;
(iii) Likely carriers: mothers with disseminated pathology or siblings of the patient.
(2) Application of genetic diagnosis to detect DMD lesion gene carriers, prenatal genetic examination of pregnant gene carriers, if the fetus is found to be DMD or BMD, abortion should be performed to prevent the birth of the affected child.
3, prognosis: typically in adolescence, severe disability, long-term toe walking Achilles tendon contracture, generally to 9-12 years of age when the child walking function disuse can make the elbow, knee contracture, most children with myocardial involvement, a small number of children with severe damage to the occurrence of congestive heart failure; about 20 years of age, respiratory distress, late need to assist breathing. Patients tend to die of respiratory infections, heart failure or wasting disease before the age of 25-30 years.
Progressive myotonic dystrophy – nursing care
Principles of myotonic dystrophy care.
(1) Keep the environment clean and quiet, pay attention to moisture and cold, actively prevent and treat respiratory infections and other complications.
(2) Adhere to physical exercise, self-massage to increase activity, promote blood circulation, prevent muscle atrophy, but should be moderate, do not overwork.
(3) The diet should be light and nutritious, avoid eating greasy, thick and overheated food, injuring fluid and damaging the spleen and stomach. At the same time to ensure nutrition, weight control should be appropriate.
(4) Actively fight against the disease, adhere to appropriate recreational activities, and encourage patients to establish an optimistic and cheerful mood, and establish confidence to overcome the disease with strong perseverance.
Family care.
(1) Create a good environment for the patient in terms of spirituality, maintain reasonable expectations, and avoid overprotection.
2, diet should be high protein, rich in vitamins, calcium, zinc, lean meat, eggs, fish, shrimp, animal liver, ribs, fungus, mushrooms, tofu, cauliflower, etc. can be appropriate to eat more, less or avoid eating too spicy, too salty, cold and other indigestible and stimulating food.
3.Adopt the exercise within your ability, and do not overwork.
4, the upper limbs can practice lifting, push-ups, chest expansion, etc.; waist can practice sit-ups; lower limbs can practice squatting, stairs, jumping, side leg press, etc.; pay attention to prevent contracture, the knee joint, Achilles tendon joint after hot compress appropriate traction; pseudohypertrophy parts of the massage to knead method; prevent spinal deformity Keep a good sitting posture, after exertion should lie down to rest.
5, in view of the progressive aggravation of the disease, the disability rate is high, so early treatment, control the development of the disease, can improve the quality of survival, especially in the family history of similar diseases, more attention should be drawn to early examination and diagnosis.
6, during the treatment period, avoid smoking and alcohol, spicy and salty food, avoid wind and cold, prevent cold, drink more water, eat more food containing calcium and zinc, maintain a relaxed mood, appropriate exercise, the patient’s family should cooperate with massage, the patient himself should overcome difficulties, adhere to appropriate exercise.