In Landouzy-Dejerine also known as the Facioscapulohumeral Muscular Dystrophy, the facila and the shoulder muscles become weak and it thus becomes diffiuclt to drink with straw, or to whistle,etc. There is no specific treatment for Facioscapulohumeral Muscular Dystrophy. But there are some treatments relative to Facioscapulohumeral Muscular Dystrophy which can give relief to some extend but they won’t cure the disease as a whole. Along with these treatments, there are some devices which patient can use to make the symptoms less severe. Surgical and mechanical help, Orthoses, exercise and diet are some of the treatments for Facioscapulohumeral Muscular Dystrophy which help the patient to fight with this disease. Orthoses help the patient with the weakening muscles in the upper and lower back. Orthoses recommend the patients to use several devices such as back supports, corsets, girdles and special bras. Surgeries are done to treat the disease in the shoulder region. It includes attaching shoulder blades to ribs. Even though Facioscapulohumeral Muscular Dystrophy patients deal with muscle loss some of the moderate exercises can be done which are useful and never hurt them. Exercises that don’t cause muscle cramp, extreme fatigue or any other pain can be practised. It’s better to take advice from the physical therapist before practising these exercises. There is no common diet for Facioscapulohumeral Muscular Dystrophy, patients should take their diet according to the doctor’s prescription. All these diets should be taken under medical supervision.
Scapular stabilization is the best way to treat Facioscapulohumeral Muscular Dystrophy. Scapular stabilization is used to treat severe scapular instability. The patient who is already suffering from weakened shoulders and abductors will lose their effectiveness due to scapular instability. Scapular fixation fixes the scapula which is moved into a stable position. For the functioning of the intact ball and socket muscle, it creates a platform which is the ease of achieving overhead positions, less fatigue and winging impossible.
Acceleron pharma’s treatment ACE-083’s3 has the ability to treat Facioscapulohumeral Muscular Dystrophy. The treatment results in increasing the muscle mass in the face and in other infected areas. The treatment ACE-083 restraints the proteins which include Myostatin. Myostatin is a one which combines with another protein called transforming growth factor (TGF)-beta which results in the reduction of muscle strength. Both the muscle mass and strength can be increased by the treatment ACE-083’s3.
Exercises such as standing rows, dynamic T and I, Bilateral external rotation, Modifies plank with protraction, Pec Stretch, W and snow angel on Foam roller can be used to scapular stabilization in shoulder muscles. Standing rows include a resistant band tied to an anchor. Patient has stretch the band until the band has a mild tension and then he has to bend back and down. In the dynamic T and I method the patient has to lay upside down on the floor with the hands stretching outside like T and then those hands to be brought nearer to his legs so that it will look like I. In bilateral external rotation method the patient has to stand up straight in 90 degrees by holding a resistant band in his hand. He has to stretch the bands in both left and right direction at the same time. This has to be repeated for 10 counts. Modified plank with protraction involves taking push-ups by resting the elbows on the floor. For each push up the patient has to hold for 3 seconds and this has to be repeated for 10 times. In T, w and snow angel method the patient has to position them on a foam roller and then stretch their hands like T, W and snow angel. In this postures, the people should not let their hands themselves to touch the ground until they are attracted due to gravity.
The patient who wants to undergo this surgery should have poor shoulder function and they should have the desire to get better from it. The patient should not have any significant medical concerns. The patient should be affected around area involving scapula muscles such as normal rotator cuff and deltoid. Patient has to respond to the manual compression test conducted by the doctors so as they can proceed forward towards the process. At last, the patient who is willing to proceed to surgery and obeys with restrictions before the operations are allowed to take the treatment.
People under 14 years of age are not eligible to undertake this treatment. People affected by this disease in their body parts other than shoulders are also cannot take this treatment. Money is an important factor as this treatment is costlier. People who don’t have enough money also should not undergo this treatment as the treatment is a continuous process for one year and cannot be stopped in the middle. Discontinuing the treatment will result in severe problems.
There are no side effects to the patients who are undergoing this treatment. The patient should follow the advice given by the doctors regularly. Proper treatment and regular examinations are musts. Exercises which involves recreational activities, repeated shoulder and arm movements should be done regularly as per the advice was given by the physical therapist. Improper exercises or exercises which are not recommended by the doctors can cause severe pain and sometimes results in failure of the surgery.
Post-treatment may vary according to the patients. Anaesthesia will be given to the patients who feel pain for several days. Lungs will be checked for puncture in the chest tube, blood and shortness in breath. After that Rehab protocol will be followed for the first 42 days motions using hand should not be done. Should involve in any type of pool activities. After 63 days the bones will start to heal then aquatic therapy such as motion in water can be done. This Rehab protocol should be followed for a duration of 19 weeks. After 19 weeks the shoulders will get attached or fused to ribs. Hence the patient can increase his activities up to which he can tolerate.
In case of various complications certain surgeries can be carried out. It takes around 1 year to fully recover after the surgery. The surgery time will be around 3 to 4 hours. After six weeks of surgery, the patient may able to do some passive motion across the shoulder. Between the weeks 8 to 12, the bone may heal and the patient may do slight muscle work like feeding, dressing etc. After 18 months patient can be in full active motion. After six months the patient can do his normal work but be careful for a period of one year in doing in motions.
Price for the treatment of Facioscapulohumeral Muscular Dystrophy varies. According to the reports, the average medical cost may be around 14 lakh RS and the average non-medical cost will be 8 lakh RS. So a total of 25 lakh RS will need to take this treatment in India. If the patient is diagnosed with this disease at the starting stage then there is no need for surgery and can be treated with a physical therapist. Cost of the treatment with the physical therapist will lower when compared to treatment with surgery. It may cost up to 5 lakh RS in India.
Results of this treatment are permanent. People can be back to their normal life after the period of one year. People should withstand all the pain for this period. After that people should follow their regular course of medicine, diet and exercise. The result will be permanent but the cure will not be 100%. The treatment will help the patient to keep going with the disease.
ACE-083’s3 is a treatment done by Acceleron pharma. It serves as an alternative for scapular stabilization. Acceleron pharma’s treatment ACE-083’s3 has the ability to treat Facioscapulohumeral Muscular Dystrophy. The treatment results in increasing the muscle mass in the face and in other infected areas. The treatment ACE-083 restraints the proteins which include Myostatin. Myostatin is a one which combines with another protein called transforming growth factor (TGF)-beta which results in the reduction of muscle strength. Both the muscle mass and strength can be increased by the treatment ACE-083’s3. The main disadvantage of this treatment is that it is still in the developing stage. When completed it can be used as a complete replacement of Scapular stabilization which can even cure more than this.