Sickle Cell Information Center Protocols

by James Eckman, M.D. and Allan Platt, PA-C

Substance Abuse and Addiction

Despite many opinions to the contrary, sickle cell anemia does not predispose to or provide absolute protection from drug addiction. A small number of patients exhibit behavior diagnostic of narcotic addiction. These patients have very frequent contact with health professionals which both compromises their health care and that of other patients with sickle syndromes. Treatment of patients with drug addiction and a sickle cell syndrome poses a number of very difficult management problems. First, drug addiction compromises our ability to diagnose and treat complications in the disease. Pain episodes are less responsive to therapy in drug tolerant patients. Drug seeking behavior may mask symptoms of serious complications. Second, regular use of opiates may make the health of sickle syndrome patients worse. For example, regular meperidine use is associated with seizures. Also, drug withdrawal may precipitate severe acute pain episodes and other more life-threatening complications.

Sickle cell syndromes also complicate the treatment of narcotic addiction. It is not realistic to expect these patients to achieve the goal of many drug treatment programs which is total narcotic free status. Pain episodes may require administration of opiate analgesics on an infrequent basis for a short period of time, even in the setting of know addiction. Some patients may have complications that cause chronic pain which will be unmanageable with non-opiates once the individual has become use to the relief obtained by regular opiate use for prolonged period of time.

Addiction in the sickle cell patient must be addressed with a positive and constructive approach. Principles established in the treatment of addiction are incorporated into an individual treatment plan that takes the individual’s strengths, problems, and needs into account. This treatment plan forms a basis for a formal contract which defines the treatment plan, health care givers and patient responsibilities, treatment alternatives, and consequences of non-compliance. Although it is acknowledged that many patients in this addicted group do not want specific treatment for addiction, a positive, constructive approach is required to provide adequate comprehensive medical management of patients with sickle syndromes and iatrogenic drug addiction.

Present Illness Document the types of analgesics used with documentation of drug of choice, level, pattern, and length of use. Allergies need documentation by symptoms. Specific criteria which establish a pattern of addiction should be sought including frequency and duration of use, drug seeking behavior, evidence of physical and psychological dependency, and disruption of normal functioning by drug use. Document the primary opiate of choice and any other analgesics or street drugs used and outline frequency of such use, routes of administration, situations when drug is used for other than pain control, and situations when the drug is not taken as prescribed. Document the year and age of first use of the drug of choice and any other psychotropics. Document reactions to the drug of choice including local reactions, sterile abscesses, seizures, hallucinations, and dysphoria. Evidence for physical dependence will sought by documenting opiate withdrawal symptoms such as yawning, nasal congestion, rhinorrhea, abdominal cramps, nausea or vomiting, dizziness, diarrhea, headache, sweating, and generalized pain in the joints. The longest period of time that the patient has not received any opiates in the past three months will be determined. Evidence for psychological dependence will be documented by defining drug seeking behavior, anxiety about being without medications, and fixation on a drug of choice. Disruption of normal functioning will be estimated by documenting amount of time per week the patient spends in treatment, the time spent gainfully employed, time spent caring for family or being a housewife, the time spent for schooling, the time spent in social functions outside of the clinic, the time for entertainment, and the time spent sleeping.

Psychologic History Evaluation by a psychologist or psychiatrist is beneficial to determine the need for treatment of underlying psychological problems or psychiatric illness. Coping strategies and support systems can be defined.

Social History Complete social evaluation is also very important to determine the patients ability to function, support systems in family and friends, and activities which will provide diversion during treatment.

- A complete physical examination is down to define conditions that cause chronic pain, complications of sickle cell and drug abuse.

- Minimum Lab. CBC with reticulocyte and platelet count, urinalysis, urine drug screen, and Chem pannel are done. Chest x-ray, PPD, VDRL, and HIV are considered essential.

- Additional Lab. Radiologic studies are obtained to define pathology in areas of chronic pain. Other tests are ordered as indicated by results of history, physical and other tests.

- Drug Addiction. Diagnosis should utilize the criteria outlined in the Diagnostic and Statistical Manual - IV. A maladaptive pattern of substance use leading to clinically significant impairment or distress as manifested by three (or more) of the following, occurring at any time in the same 12-month period:

Substance is often taken in larger amounts or over longer period than intended

Persistent desire or unsuccessful efforts to cut down or control substance use

A great deal of time is spent in activities necessary to obtain the substance (e.g., visiting multiple doctors or driving long distances), use the substance (e.g., chain smoking), or recover from its effects

Important social, occupational, or recreational activities given up or reduced because of substance abuse

Continued substance use despite knowledge of having a persistent or recurrent psychological, or physical problem that is caused or exacerbated by use of the substance

Tolerance, as defined by either: need for read amounts of the substance in order to achieve intoxication or desired effect; or markedly diminished effect with continued use of the same amount

Withdrawal, as manifested by either: characteristic withdrawal syndrome for the substance; or the same (or closely related) substance is taken to relieve or avoid withdrawal symptoms.

Many of these manifestations may also be manifestations of sickle cell disease, appropriate treatment of pain, and inadequate pain management. I is critical that the behavior is caused by drug abuse, that is, use not related to pain control.

- Chronic Pain From Sickle Complications. Although most of the pain in sickle cell is acute and episodic, certain conditions like aseptic necrosis, bone infarctions, and sickle arthropathy may cause chronic severe pain. Some patient appear to need chronic opiate pain medications to deal with this pain. These patients will exhibit withdrawal, increasing tolerance, and may experience decreasing function from chronic pain and regular appropriate opiate use that may be inappropriately diagnosed as addiction.

- Drug Addiction. Treatment plans should all have the following elements.

- Detoxification over six weeks or less as inpatient or outpatient by gradual reduction in opiates taken on a regular basis.

- Every patient is expected to gain and maintain opiate free status as an outpatient.

- If chronic daily opiate treatment is desired by the patient, this is provided through an established drug dependency unit using their guidelines.

- Patients may be evaluated and treated for pain episodes and other problems evaluation at any time, however, treatment for routine crisis includes hydration, NSAIDs, and opiate analgesics as reguired to control the pain acutely.. When needed, opiate medications are given on a regular schedule. Use of opiate analgesics as an outpatient is avoided, if possible, in these patients. Opiates of the mixed agonist/antagonist class are never used for pain episodes if the patient is on regular opates. The patient’s past drug of choice should not be used again for the treatment of pain episodes.

- Compliance to treatment upon completion of detoxification is monitored by random urine and serum drug screens.

- Appropriate supportive services, including group and individual counseling sessions, should be made available to each patient. Effort will be required to offer substitute activities to replace the loss incurred by being treated regularly. Vocational rehabilitation is important. All patients should be encouraged to attend weekly meetings with an established support group such as Narcotics Anonymous or Alcoholics Anonymous. Patients who are successful with detoxification and maintenance should be asked to assist in the rehabilitation of other patients.

- Each patient’s medical problems should be managed concurrently with and individualized treatment plan.

Nursing Considerations

 Observe for signs/symptoms withdrawal. If the patient is in withdrawal, maintain a quiet, safe environment, remove harmful objects, use restraints if ordered by physician, side rails up, reassure the anxious patient that medications will control withdrawal symptoms.

 Monitor and promote adequate nutritional intake/administer medications carefully to prevent hoarding by the patient

 Refer to detoxification/rehabilitation appropriately as recommended.

 Educate patient to treat chronic pain with nonopiate analgesics and therapy.

 Patients can get into trouble because pain medications are taken to alter mood instead of to manage pain

Provide education to patient and family about management of stress, sleep disturbance, emotional distress and avoidance of preventive medication for anticipated painful episodes.

Refer to social worker or psychiatry as needed

The clinical heterogeneity of sickle cell disease requires a graded approach to pain treatment. There is no single drug that is appropriate or best for every sickle cell patient or for each painful episode in a given patient. The choice of analgesic for each pain episode should be based on the prior history of pain, previous drug use, complications from sickle cell disease, and a current historical and physical assessment of the pain. Certainly patients experiencing acute, moderate to severe, and severe pain should be treated with a opiate by the parenteral route on a regular schedule. As soon as the patient shows a significant and sustained improvement, the opiate drug should be taper and stopped. Treatment during recovery includes NSAIDs and less potent opiates such as codeine and hydrocodone for short periods, less than a 48 hour supply.

Chronic pain should only be treated with opiates in selected patients. Chronic pain can be managed with NSAIDS, plain acetaminophen, relaxation therapy, or other behavioral approaches. Nerve blocks and TENS may assist in the control of localized pain such as rib infarcts or leg ulcers. When chronic treatment with opiate analgesics is required to control pain, long-acting preparations should be used.

In our experience, most patients develop problems related to drug dependency for a number of reasons. The most common problems are inappropriate treatment of any pain with strong opiates and abuse of the pain medication by taking opiates for non-pain related problems. Patients and parents must be educated to learn to evaluate the type and level of pain and use a graded approach to management. Non-opiate analgesics should be used for the common pains headaches, muscle and joint pains, chronic pain, premenstrual pain, and dysmenorrhea. Behavioral and adjuvant measures are stressed for all types of pain. Education about abuse of all psychoactive substances needs to be stressed. Alcohol and cocaine must be avoided because of their tendency to regularly precipitate pain episodes and more serious complications. Many patients get into trouble because they start taking their pain medications for the mood altering potential. Education includes advice about avoiding use of pain medications for stress, sleep, anxiety, or to "prevent" pain episodes.

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Copyright © 1997 Sickle Cell Information Center
Last modified: June 12, 2001