Treatment of epidermolysis bullosa aims to prevent complications and ease the pain of the blisters with appropriate wound care. The condition often progresses despite treatment, sometimes causing serious complications and death.
Working with a treatment team
Addressing the many aspects of wound care usually requires a multidisciplinary approach. Members of your care team may include:
- A primary care physician who oversees the treatment plan
- A physician specializing in wound care
- Nurses or medical assistants who provide both care and education for managing wounds
- A physical therapist and occupational therapist
- A nutritionist
- A social worker
Sometimes your doctor may involve other specialists, such as a surgeon, a dermatologist, an eye doctor (ophthalmologist), a dentist, a psychologist and a geneticist.
Medications can help control pain and itching and address complications such as infection in the bloodstream (sepsis). Prescription oral antibiotics may be used if you show signs of a deep or widespread infection (fever, weakness, swollen lymph glands).
An oral anti-inflammatory drug (corticosteroid) may help reduce the pain from dysphagia (difficulty swallowing).
Surgery may be considered to correct normal motion or to improve your ability to eat a healthy diet.
- Restoring mobility. Repeated blistering and scarring can cause deformities, such as fusing of the fingers or toes or abnormal bends in the joints (contractures). Your doctor may recommend surgery to correct these deformities, particularly if they interfere with normal motion.
Widening the esophagus. Blistering and scarring of the esophagus may lead to esophageal narrowing, making eating difficult. Surgical dilation of the esophagus can relieve this and make it easier for food to travel from the mouth to the stomach.
The patient receives light sedation, then the surgeon positions a small balloon in the esophagus and inflates it to widen the area.
- Placing a feeding tube. To improve nutrition and help with weight gain, a feeding tube (gastrostomy tube) may be implanted to deliver food directly to the stomach. Feedings through the tube may be delivered overnight using a pump. Eating through the mouth is continued if possible so that the child will be able to eat with others for normal socializing.
- Grafting skin. Several types of skin grafts have been used to treat wounds from epidermolysis bullosa. The Food and Drug Administration has approved a type of composite cultured skin (OrCel) for this purpose.
Working with a rehabilitation specialist (physical therapist, occupational therapist) can help ease the limitations on motion caused by scarring and shortening of the skin (contracture). A rehabilitation specialist can also give you guidance on the best ways to keep you or your child safe while going about daily activities. Swimming may also be helpful in preserving or regaining mobility.
Researchers investigating treatments
Intensive studies are underway to find better ways to treat and relieve the symptoms of epidermolysis bullosa, including:
Aug. 22, 2014
- Gene therapy
- Bone marrow transplantation
- Protein replacement therapies
- Cell-based therapies
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