Pain is reported by people with all types of EB. Complaints of skin and wound pain from active blisters and sores are most common. Other complaints stem from corneal (eye) blisters, bone and dental/oral pain. It seems the most severe pain is reported during dressing changes, baths and bowel movements — activities that occur on a daily basis.
First, the cause of the pain should be identified. For example, if the pain is the result of a wound infection, seeking medical attention will address the pain. If the pain is from a corneal blister or erosion, “tincture of time,” remaining in a darkened room, eye compresses and pain medication will help. If the pain is related to bowel movements, the goal is to soften the stools, which may require medication and consultation with the practitioner.
When the pain occurs regularly and is due to open wounds and dressing changes, the first step is to evaluate the bandages to be certain they are of the non-stick, atraumatic variety. If pain persists and interferes with the patient’s ability to perform activities of daily living (ADLs), then a pain-management plan is necessary. A pain-management plan begins with an evaluation of the pain. Using a validated pain scale is helpful.
A pain scale is a tool for evaluating the severity of pain, which is necessary for achieving pain relief or pain control. There are several validated pain scales. Among them is the UCLA Pain Assessment Tool (below), which incorporates the Wong-Baker FACES pain scale.
This scale is helpful because it classifies pain as mild, moderate or severe, which are terms used when developing a pain-management plan. The World Health Organization has developed an analgesic (pain relief) ladder that categorizes pain according to these levels. Recommendations for pain management are included with the appropriate ladder, as seen here:
Level 1: Mild Pain
Level 2: Moderate Pain
Level 3: Severe Pain
|Over-the-counter non-prescription meds such as acetaminophen and non-steroidal anti-inflammatory medications (ibuprofen)||Prescription narcotics (opioids) such as codeine or hydrocodone. May also be combined with acetaminophen (Tylenol #3 or Vicodin)||Prescription narcotics (opioids) such as Morphine, Hydromorphone or Methadone|
Adjuvant therapy refers to a medication or treatment used to assist or enhance the effectiveness of another treatment. Some adjuvant medications are antidepressants, such as Elavil®, anticonvulsants, such as Neurontin® and Tegretol®, or the NMDA receptor antagonist, Dextromethorphan. Meditation, biofeedback, distraction, hypnosis and visualization are other non-medication adjuvant therapies.
Addiction is a concern with regard to opioid use. Addiction is a psychological dependence and a compulsion to use drugs for non-medical purposes, not for physical pain. Physical dependence is a concern for some and describes an affect on the body that may occur if taking opioids for a period of time. In such cases, the body adapts to the medication, and if the medication is abruptly discontinued, side effects such as nausea, cramps and restlessness may occur. These effects may be minimized by tapering the medication when it no longer is needed. Tolerance describes the process the body undergoes as it adapts to using opioids (and some other medications). Often, if a person becomes tolerant of a medication, higher doses or the addition of adjuvant medications are required to achieve sufficient pain relief.
For these reasons, many patients and health care practitioners exercise caution with opioids. For people with EB, itching and constipation seem to be most troubling. Also, many find the associated drowsiness unacceptable. But when faced with pain that interferes with one’s ability to function as independently as possible, opioids may be necessary. In these situations, working with a pain-care specialist or pain-management team is highly recommended. A pain-care specialist is a physician or nurse who specializes in managing pain and achieving a level of relief with minimal side effects.
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