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Watching
a loved one with NBIA in pain is very difficult, especially when the exact
source of the pain isn’t known because the person can’t tell you.
Knowing how best to treat that pain can be even harder. My
son, Austin, is now 13, but more than three years ago, physicians believed
that we were “at the end of the road” and that Austin was certainly
near death. During the summer of 2000, he was in tremendous pain from
dystonia in his back. Our doctors also said he had extreme bouts of
inspiratory strider, (difficulty
breathing due to tightening of the muscles in the throat area), as a
result of dystonia in his vocal cords.
At times, he felt like he was suffocating. After
nearly a month in the hospital, the pain management team consulted with us
and advised us that morphine was the only way to relieve his pain. We had
certainly tried everything else at our disposal. The physicians suggested
a morphine drip. A
local hospice organization has been managing Austin’s pain with morphine
ever since. Baclofen, phenobarbital and valium are still part of his
medical regimen, but I believe morphine has kept him from suffering the
extreme pain that he had experienced before we began this treatment. As an
added benefit, Austin has gained about 30 pounds and grown in height
because of his increased comfort level. We
also have added normal saline treatments and Atrovent to his respiratory
regimen to alleviate breathing difficulties. When necessary, hospice
recommends a low dose of Roxanol be added to his breathing treatment to
prevent the feeling that he is suffocating. While
we are not sure if Austin will ever be morphine-free, we are thankful for
the many days that he has been fairly comfortable. Typically,
when NBIA patients suffer from pain, caregivers contact primary care
providers, often the patient’s neurologist. Although the doctor may feel
that morphine or Roxanol may be necessary, the physician might not feel
comfortable dosing to the level necessary to reach pain-free status. At
that point, a pain management specialist or a hospice program are options.
Frequently, pain management specialists connected with children’s
hospitals are familiar with narcotic levels and dosage amounts for
pediatric patients and patients of rare disease. Contacting hospice does
not mean that caregivers are preparing their loved ones to die. It simply
means they are accessing an important resource to provide comfort. It
is important that before morphine is used the patient
“receive an exhaustive evaluation for a treatable cause of the
pain,” said Dr. Susan Hayflick, geneticist and leading expert in NBIA at
the Oregon Health & Science University. “Too often these are missed
(a broken bone, bleeding stomach) and just the pain is treated.” If
an examination fails to reveal the cause of the pain, caregivers or
patients must ask more difficult questions. Has every diagnostic tool been
used and are we ready to treat the pain in the absence of understanding
the cause? If the caregiver were in pain, would he or she not turn to the
other person in the room and demand some sort of pain medication? Do the
patients with NBIA deserve any less? “Worsening
pain does not indicate that the person is nearing death,” Hayflick said.
“Pain episodes seem to come and go for most patients. Sometimes there is
a treatable reason and sometimes not. I think there is little, if any,
correlation between worsening pain and worsening disease.” Many
caregivers wince at the thought of morphine. They might associate the drug
with end-of-life care or worry whether there will be anything stronger if
needed later. Will the morphine hasten their loved one’s death? Dr.
Deidre Woods, medical director of Hospice of Naples, Fla., said morphine
is not necessarily reserved for end of life nor does it bring on death. “In
fact, patients frequently express a new lease on life and are able to eat
or sleep comfortably now that they are pain free,” she said. “Roxanol
use in nebulizer treatments also can help a patient to breathe much easier
especially when the patient suffers from the debilitating feeling of
suffocating due to inspiratory strider. This brings a great deal of
comfort to a patient and they can lead more productive and fulfilled lives
with morphine as part of the medical treatment program.... If the patient
requires less medication, we can decrease the amount of morphine until the
patient is comfortable.” Woods
said that respiratory problems occur mainly in rare cases where
extraordinary high doses are given, especially if the patient has never
had these types of drugs before. It is important to start at very low
doses, then increase the medication level as needed. The
use of morphine in NBIA patients is a subject of debate and will continue
to be a topic of consideration as long as our patients continue to suffer
from intense bouts of pain. While we hope for a cure, many of us will
continue to seek out information on pain management. Morphine may not be
the only answer, but for some NBIA patients, it is an important part of a
regimen of medications that attempt to keep them pain-free. |