Sickle Cell Disease

	What Is Sickle Cell Disease?
	Who Gets Sickle Cell Disease?
	Common Symptoms of Sickle Cell Disease
	What You Can Do to Help
	Treatment for Sickle Cell Disease
	Pain Management
	Helpful Sickle Cell Disease Resources

Caring for a loved one with sickle cell disease is no easy task. Not only does it require caring for someone you love at home, but it also means facing your own concerns about the disease. It may be frightening to discover that there is no cure, and you may worry about other family members becoming ill with sickle cell disease.

Physical and emotional care can be time-consuming and exhausting. Many caregivers of people with sickle cell disease have concerns about their loved ones' illness and future health prospects. Practical concerns, such as worries about financial issues and time management, are also common. The goal of this chapter is to address some of these concerns.

Learning more about sickle cell disease may help you feel more at ease in your caregiver role. This can be important for new caregivers of small children with the disease. The feeling of helplessness from caring for a sick child or relative of any age can be reduced by becoming knowledgeable about the disease. It can be difficult to learn about a disease when you do not know where to start. This section provides information about sickle cell disease, including what caregivers can do to help their loved ones.

What is Sickle Cell Disease?

Sickle cell crises

Factors That May Cause a Crisis Having an infection, such as a cold or the flu Cold weather Being overly tired or fatigued Exercising too hard Being dehydrated (not drinking enough water and other fluids) Not having enough oxygen in the blood

Who Gets Sickle Cell Disease?

Common Symptoms of Sickle Cell Disease

Children may start to have symptoms of sickle cell disease as young as 6 months of age. Babies who are suffering from sickle cell symptoms may be irritable or cranky, even when their parents do everything possible to provide comfort. Also, children with sickle cell disease may develop more slowly and have difficulties in school because of the pain from the disease. Parents who learn about the disease are more likely to be able to help their children.

The most common symptom of sickle cell disease is pain. During a crisis, pain may seem to come from the bones, usually in the arms, hands, legs, feet, or back. Also, there may be pain in the stomach or chest.

Other health problems for people with sickle cell disease include:

	Infection
	Anemia (or a low blood count)
	Fatigue
	"Acute Chest Syndrome" (severe chest pain with breathing difficulties)
	Eye problems such as bad eyesight and blindness
	Skin ulcers or skin sores that do not heal well, especially on the lower legs
	Jaundice, or yellowing of the whites of the eyes or skin
	Damage to the organs, such as the liver, kidneys, or lungs
	Brain damage or stroke

What You Can Do to Help

	Learn as much as possible by reading and asking experts about sickle cell disease.
	Find medical practitioners who understand the disease.
	Ensure regular visits to a family doctor, pediatrician (children's doctor), a hematologist (blood specialist), and/or a specialized sickle cell clinic.
	Ask questions of your loved one's doctors and discuss your concerns.
	Help the patient avoid factors that bring on crises.
	Find ways to avoid and manage stress in the household.
	Make sure that the patient drinks plenty of fluids.
	Administer pain medications as prescribed.
	Offer to massage sore or painful areas for your loved one, if the physician approves.
	Encourage your loved one to use relaxation techniques and other pain management strategies. Here is more information on relaxation techniques.
	Watch for signs of a sickle cell crisis.
	Help the patient rate and record their pain and fatigue in a daily journal.

Here is more information on pain, fatigue, and difficulty breathing.

Treatment for Sickle Cell Disease

General medical treatments

These general medical treatments are recommended by physicians:

	Antibiotics – medication given to prevent infections
	Erythropoietin – medication given to improve anemia (low blood count)
	Blood transfusions – increases blood count to improve anemia
	Hydroxyurea – medication given to reduce the chance that the red blood cells will change into the sickle shape
	Folic acid – a vitamin which helps to treat anemia
	Hydration (increasing the level of fluids in the patient's body) – counters the frequent problem of dehydration (decreased level of body fluids)
	Oxygen – provided via an oxygen mask, nasal catheter (tube connected to nose), or oxygen tent, increases the level of oxygen in the blood
	Treatment for pain – drug and non-drug approaches

Preventing crises

	Limit intake of alcohol and avoid smoking.
	Keep warm and avoid cold or rainy weather.
	Get plenty of sleep and rest, and let the patient's health practitioner know about any difficulties with sleep.
	Exercise regularly, but not too hard.
	Drink plenty of fluids (at least 8 glasses of water a day in warm weather).
	Learn to manage emotional and physical stress effectively.
	Treat infection immediately.
	Monitor pain medication use with a trained medical practitioner.

When to call the doctor

Crisis Warning Signs

	Fever of 101 degrees Fahrenheit (38 degrees Celsius) or higher
	Pain not relieved by medication
	Shortness of breath or fast breathing
	Severe headaches or dizziness
	Severe stomach pain or swelling
	Jaundice (yellow color in whites of eyes or skin) or very pale skin
	Painful erection in males
	Sudden change in vision
	Seizures
	Weakness

Children and Adults Who Have Difficulty Communicating

Watch For These Signs of a Sickle Cell Crisis: Changes in breathing pattern Frequent coughing Unusual crankiness or crying Screams or sudden movements when touched Loss of appetite Vomiting or diarrhea Changes in urination Unusual tiredness or weakness Swollen hands or feet Pale blue or gray lips or skin>

Here is more information on how and when to effectively communicate with your loved one's health care team.

Pain Management

When a sickle cell crisis is severe, patients often go to the emergency room. Sickle cell patients sometimes have bad experiences in emergency rooms and hospitals when health care professionals question their pain and their need for medications. Some may be labeled as "addicted" to their pain medications. You should know that wanting medications to help the severe pain of a sickle cell crisis does not make a patient a drug addict, or mean that they are trying to "get high." These bad experiences may lead sickle cell patients to distrust health care professionals. It is important that patients be able to find health practitioners whom they trust. You can help by locating a health care team that is knowledgeable about sickle cell disease and sickle cell pain. Getting to know the members of the team and making sure they know your loved one is also important.

Still, you and other family members may worry about addiction to pain medications, particularly opioids (also called narcotics). It can help to understand the difference between addiction and physical dependence:

	As tolerance increases the drug becomes less effective and higher doses are required.
	Physical dependence means that a person will have symptoms of withdrawal when the drug is suddenly stopped.
	Addiction occurs when drugs are used compulsively for reasons other then pain relief, and when their use is continued despite harm.

Non-Opioid Medications Prescribed for Pain Hydroxyurea (Hydrea and Droxia are examples) Anti-inflammatory medications (such as aspirin, ibuprofen, or Vioxx) Steroids (such as prednisone) Tricyclic antidepressants (such as amitriptyline) Anticonvulsants (such as gabapentin)

Non drug treatments

	Transcutaneous electrical nerve stimulation (TENS) – a low intensity electrical current can sometimes be effective for pain relief.
	Physical therapy – gentle exercises and heat and cold treatments can help with sickle cell disease pain.
	Relaxation techniques – learning how to relax can help patients cope better with the illness and pain.
	Massage – especially at the first signs of a crisis, massage can decrease muscle stiffness and potentially reduce pain.
	Acupuncture and acupressure – can be useful in helping to relieve stress and manage pain.
	Distraction – engaging activities (such as hobbies, video games and movies) that change the patient's focus can help relieve stress and pain.
	Psychotherapy – speaking with a mental health professional about the stress and frustration of sickle cell disease and/or learning cognitive/behavioral techniques for coping with the disease can be beneficial for both you and the patient.

How you can help obtain treatment for pain

	Find medical practitioners who understand your loved one's sickle cell-related pain.
	Ensure regular visits to sickle cell pain specialists.
	Ask questions of doctors and discuss your concerns.
	Administer pain medication as prescribed.
	Offer to massage sore or painful areas if the patient's physician approves.
	Encourage the use of relaxation techniques to relieve stress and decrease pain. Here is more information on relaxation techniques.
	Find ways to avoid and manage stress in the household.
	Distract your loved one with activities he/she enjoys.
	Watch for signs of pain or other discomfort.
	Help the patient rate and record pain in a daily pain journal.
	Contact the patient's medical practitioner if pain is not relieved by medication.

Here is more information on pain.

Helpful Sickle Cell Resources

Organizations

Websites

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