Sickle Cell Transition Education Project (STEP)

STEP UP, the Sickle cell Transition Education Project, is a just for teens with sickle cell disease to help them better understand his or her health condition. Teens will learn how sickle cell is affecting their bodies, how to make the move from pediatrician to an adult doctor, how to manage pain, learn about sexual health, and also ask questions and learn from other teens with sickle cell disease.

Transition

Transition

What is transition?

Transition means changing or moving from one stage or place to another. Everyone goes through transitions in life - like when you graduated from elementary school. Transition can be exciting, involving new experiences. But, transition can also be difficult and scary, especially when you are not prepared for it.

An important transition in your life will be moving from child to adult-centered healthcare for your sickle cell disease. This transition occurs differently around the country depending on your sickle cell program, but usually involves changing your sickle cell doctor and hospital. Transition also involves other important aspects like:

  • Having a better understanding of sickle cell disease
  • Answering and asking questions at your medical appointments
  • Making your own medical decisions
  • Taking your medicines independently
  • Advocating (speaking up!) for yourself

With the help of this site, your health care team, and your family, you can have a successful transition!

When does transition occur?

Transition should not happen at any one time or age. Instead, transition should be a gradual process; it is something that you, your family, and your healthcare team work on over time.

It is never too early to start thinking about transition because preparation leads to success. It is a good idea to start creating a transition plan with your healthcare team before age 14. You will be able to work on this plan for a few years before actually moving to a different doctor or hospital.

It is important to know that some pediatric hospitals have different practices and rules regarding care of older patients. For example, at some places patients over the age of 21 cannot be admitted to the pediatric hospital. At other places the age limit is 18 or 22 years. Ask your doctor about the policies at your hospital.

Policy at Children's National Medical Center (PDF)

Where should I transition?

Talk to your healthcare team about this important question. Depending on your location you may have many options or one preferred place.

Why do I need to transition?

Transitioning allows you to become a successful, independent adult. While you might not like some of the responsibilities that come with transition, remember that you are gaining more privileges. Through transition, you earn the freedom to be in control of your own life.

Transition can be difficult because you might be leaving a pediatric doctor or healthcare team that knows you well and that you like. These individuals, however, are often not specifically trained to treat adults. Treating adults and children with sickle cell disease can be very different, and pediatric hospitals are designed to take care of children, not adults.

Get prepared

This website is designed to help prepare you to transition from the world of pediatrics to adult medicine. We hope this website will help you become your own health advocate.

What is an advocate?

An advocate is someone who stands up for a cause. As a teen with sickle cell you now need to start speaking up--advocating-- for yourself.

Listen to some young adults with sickle cell talk about advocacy.

To become a good advocate for yourself, you must practice talking to your doctor independently. 

Am I ready for transition?

Read the below statements and answer "YES" or "NO" for each one.

If you can answer "YES" to most, great, you are likely ready to transition soon. If you answer "NO," to a statement, use this website and talk to your doctor for help related to that topic.

Medical History Checklist

  • I know what type sickle cell disease (hemoglobin SS, SC, S beta thal, etc) you have?
  • I know my baseline hemoglobin level?
  • I know my baseline pulse ox level?
  • I know all of my medication names and doses?
  • I know what all of my medications are for and what they do?
  • I know all of my drug allergies?
  • I know all of the surgeries that I have had?
  • I know I have developed antibodies because of past blood transfusions?

Note: Checklists below taken from the Virginia Department of Health

Medical Knowledge Checklist

  • I understand behaviors and triggers that can cause a pain episode.
  • I know why drinking a lot of fluid is important for people with sickle cell disease.
  • I understand the importance of a healthy diet as it relates to sickle cell disease.
  • I understand how I got sickle cell disease.
  • I understand how I might pass sickle cell disease on to my child.
  • (For females) I understand how sickle cell disease could affect my health if I got pregnant.
  • I know about serious complications in sickle cell disease (acute chest syndrome, stroke, priapism, etc).
  • I know what is considered an emergency related to sickle cell disease and what to do.
  • I know how the use of tobacco, alcohol, and other drugs can affect my sickle cell disease.

Medical Skills Checklist

  • I can tell someone what sickle cell disease is and how it makes me feel.
  • I know how to use a thermometer and what to do if I have fever.
  • I answer my doctor’s questions during my clinic visits.
  • I ask questions during my medical appointments.
  • I know how to schedule a medical appointment.
  • I keep track of my medical appointments using a calendar.
  • I can get myself to my medical appointments.
  • I know how to get my prescriptions filled.
  • I have or am working on a medical transition plan when I leave pediatrics

Education and vocation skills checklist

  • I have a 504 Plan or Individualized Education Plan (IEP).
  • I participate in my 504 or IEP.
  • I plan to graduate from high school / vocational school or obtain a GED.
  • I have been thinking about the kind of career I would like as an adult.
  • I have talked to my school counselor or a teacher about my educational / career goals.
  • I know what type of training I might need for the career I want.
  • I know the types of work situations that could cause problems related to sickle cell disease.
  • I have practiced my job interview skills.
  • I know how to write a resume.
  • I know where to find information about job training and opportunities.
  • I know where to find information about educational scholarships.
  • I have talked with my parents about my vision for my future.

Health benefits checklist

  • I know what kind of medical insurance I have.
  • I understand the different types of health benefits that are available to me.
  • I know how my age can affect my health benefits.
  • I carry my own copy of my health insurance card.
  • I have a plan for the future regarding my health benefits.

Social support checklist

  • I have a good support system of family and/or friends.
  • I understand what self-esteem is.
  • I understand the difference between being sad and being depressed.
  • I understand what "healthy relationships" are.
  • I have hobbies or activities that I enjoy.
  • I have a person that I can talk to about sickle cell disease.
  • I have a positive vision of my future.

Please note above checklists were adapted with permission from the Virginia Department of Health Sickle Cell Transition Intervention Program

My Body

My Body

Understand how sickle cell disease could affect your body. Since blood travels all over, sickle cell disease affects many different parts of the body. It can be scary reading about these problems, but try not to be nervous. Most people with sickle cell will not experience all of these complications. Also if you know about these issues and you understand your body, you could prevent some of these problems or get help for them before they cause you too much trouble.

1. Brain

Stroke

Defined: A stroke occurs when blood flow to the brain is blocked.

Signs of a stroke include suddenly having any of the following:

  • Numbness or weakness - especially on one side of the body
  • Confusion, trouble speaking or understanding
  • Trouble seeing
  • Trouble walking, dizziness, and loss of balance
  • Severe headache with no known cause

What should I do if I think I might be having a stroke?

Act fast. If you have signs of a stroke, immediately call 911.

Remember FAST:

Face. Smile. Does it look uneven or droop? Arms. Raise both arms. Does one side drift down? Speech. Repeat a simple sentence. Does it sound abnormal or slurred? Time. If you answer yes to any of the above questions then brain cells could be dying and you need urgent medical treatment.

What is a TCD?

As a child you should have had a test called a Transcranial Doppler (TCD) that measures the flow of blood to the brain. This test helps predict a person’s risk of having a stroke. Children who have blood flow measured by TCD faster than a certain speed are more likely to have a stroke. Many of these children are then placed on chronic blood transfusions to lower their risk of stroke. Also, individuals who have had a stroke are often started on chronic blood transfusion therapy to lower their risk of having another stroke.

2. Eyes

Jaundice

Defined: Jaundice is a word used to describe the yellow color most easily seen in the normally white part of the eyes (sclera).

People with sickle cell have jaundice because of the breakdown of red blood cells that releases bilirubin, a yellow substance. Many people with sickle cell always have some mild jaundice because their red bloods cells are broken down much more often than people who do not have sickle cell. This jaundice is not concerning.

What if my eyes are more yellow than usual?

If you have increased yellowing of your eyes (increased jaundice):

  1. Your body might be destroying an increased number of red blood cells. Contact your doctor right away if you have increased jaundice plus urine that is dark brown, like the color of cola.
  2. You might be dehydrated. Drink more fluids, especially water.
  3. You could have a problem with your liver or gallbladder. Jaundice that is due to liver or gallbladder problems is less common. However, if you have increased jaundice that does not improve with rest and fluids, talk to your doctor about liver function tests.

Retinopathy

Defined: Retinopathy is a type of damage to the back of the eye that controls vision.

People can develop different types of retinopathy for various reasons and those with sickle cell disease can develop a specific type of retinopathy (sickle cell retinopathy). People with early retinopathy have no symptoms and may have perfect vision, however, retinopathy often worsens, causing vision problems and even blindness. Fortunately, retinopathy can usually be treated by laser surgery before it causes vision problems.

People with sickle cell should see an ophthalmologist (an eye doctor) to look for retinopathy every year starting at around age 10. It is very important to see the ophthalmologist even if you have 20/20 vision because you want to treat retinopathy before it causes problems.

3. Lungs

Acute Chest Syndrome

Defined: Acute chest syndrome (ACS) is a term used to describe a type of sickle cell crisis involving the lungs. ACS is diagnosed by seeing a new infiltrate (evidence of a substance not normally present) on a chest X-ray. Some doctors may use the word "pneumonia" (inflammation of the lung) to describe this abnormality. In general, pneumonia in a person with sickle cell disease is the same thing as acute chest syndrome.

A person with acute chest syndrome has one or more of the following symptoms:

  • Fever
  • Cough
  • Difficulty breathing
  • Chest pain

It is very important to see a doctor if you have any of the above symptoms because people with ACS can become very sick.

How is acute chest syndrome treated?

Treating ACS involves helping to ensure that the person’s body is still receiving enough oxygen. Supplemental (extra) oxygen is often given. Breathing treatment medications like Albuterol or Pulmicort may also be given to help improve lung function especially if a person also has asthma. Sometimes a blood transfusion is necessary to further help with oxygen transport. Since ACS may be triggered by an infection, treatment can also involve antibiotics.

How can I prevent acute chest syndrome?

One important thing that you can do to prevent acute chest syndrome is incentive spirometry. An incentive spirometer is a device that helps encourage you to breathe in deeply. By taking deep breaths, your lungs will better deliver oxygen and prevent vasoconstriction of the blood vessels going to the lung that can cause acute chest syndrome. If you are in the hospital for a pain crisis, you should try to use your incentive spirometer 10 breaths every hour while awake, or once every time you see a TV commercial for example.

Another important thing you can do to prevent acute chest syndrome (and other problems) is to get your influenza (flu) vaccine every year. All people with sickle cell disease should get a flu vaccine before the start of the flu season in the early fall (usually first available in September). You need to get the influenza vaccine every year (unlike other immunizations which usually do not need to be repeated) because the influenza virus changes every year. The vaccine is specially made to protect against the influenza virus of the current season.

4. Heart

Pulmonary Hypertension

Defined: Pulmonary hypertension is increased pressure in the blood vessels that go to the lung for oxygen transport. This increased pressure is caused by narrowing of these blood vessels.

To help understand pulmonary hypertension, think of two hoses with the same amount of water running through them.

Hose #1 is big. Water easily travels through the hose and does not push against it (low pressure).

Hose #2 is small. Water has a more difficult time traveling faster through the hose and pushes hard against it (high pressure).

Pulmonary hypertension is a problem because it can cause a strain on the heart that has to pump blood.

Symptoms of pulmonary hypertension include:

  • Shortness of breath (dyspnea)
  • Feeling tired all the time (chronic fatigue)
  • Dizziness
  • Fainting (syncope)
  • Swollen ankles and legs (edema)
  • Chest pain, especially during physical activity (angina)

An ECHO (a heart ultrasound) can help diagnose pulmonary hypertension.

5. Spleen

The spleen is an organ in the left upper abdomen that removes old or damaged red blood cells. The spleen is also part of the immune system and helps fight certain infections.

Sequestration

Defined: Splenic sequestration is the sudden enlargement of the spleen due to trapping of red blood cells. This pooling of blood in the spleen causes problems because fewer red blood cells are able to circulate throughout the rest of the body to transport oxygen. People with splenic sequestration need to be hospitalized and may need a blood transfusion.

After recovery from a splenic sequestration event, an individual may start chronic blood transfusion therapy or have the spleen removed (splenectomy) to prevent future episodes. Splenic sequestration usually only occurs in children with sickle cell disease (before they develop functional asplenia).

Functional asplenia

Defined: The spleen in individuals with sickle cell disease usually becomes damaged over time by the many abnormal red blood cells traveling through it. Usually by adolescence, the spleen in individuals with sickle cell disease is non-functional because of this damage. Functional asplenia simply means not having a working spleen.

No spleen- who cares?

People can live without a spleen. However, people who do not have a functioning spleen have an increased risk of certain infections, especially infections from bacteria called "pneumococcus." Because of this increased risk, all people with sickle cell should see a doctor immediately if they have fever. Fever could be the first sign of a serious bacterial infection that needs immediate antibiotic treatment.

Fever = Oral temperature above 101.3° F or 38.5° C = Go to the Emergency Room (ER) or Sickle Cell Center

As a child you should have taken penicillin, an antibiotic to prevent blood infection by the bacteria called pneumococcus. It is recommended that all children with sickle cell disease less than 6 years old take penicillin everyday. It is also recommended for older individuals to continue taking penicillin if they have had a past pneumococcus blood infection or had surgery to remove the spleen (splenectomy).

6. Gallbladder

The gallbladder is an organ is the right upper abdomen that stores bile, a fluid that helps with digestion.

Gallstones. People with sickle cell disease typically can develop types of gallstones called bilirubin gallstones. Bilirubin is a yellow substance made when hemoglobin in red blood cells is broken down. People with sickle cell disease have elevated bilirubin levels because of the constant break down of damaged red blood cells. This bilirubin can then collect to form gallstones. A few gallstones may not cause any problems. However, over time, gallstones can block the flow of bile out of the gallbladder causing two problems:

  1. Biliary colic. This is the word used to describe the abdominal pain caused by gallstones. People with biliary colic can have severe abdominal pain located in the right upper abdomen. Often this pain is worse after eating fatty meals but then goes away.
  2. Cholecystitis. Cholecystitis is an infection of the gallbladder that results from blocking the flow of bile. People with cholecystitis usually have fever in addition to severe right upper abdomen pain and tenderness. People with cholecystitis need treatment with antibiotics in the hospital.

If a person is having significant problems from gallstones, the treatment is surgery to remove the gallbladder (cholecystectomy).

7. Kidney

The kidneys filter blood, removing waste to create urine.

Sickle cell nephropathy

Defined: Sickle red blood cells can damage the kidney so that it is unable to filter properly. This kidney damage is called sickle cell nephropathy. The first sign of this damage is protein in the urine (proteinuria). People with early sickle cell nephropathy have no symptoms, however, they can later develop renal failure. Renal failure means that the kidneys no longer work. Renal failure can cause dangerous levels of different electrolytes in the blood since the blood is not properly filtered. People with renal failure must go on dialysis or have a kidney transplant.

What to do if you have proteinuria?

If you have proteinuria, then you likely have early kidney damage. You should see a kidney specialist (nephrologist) to get started on medication to slow down this damage to the kidney.

8. Bone

Avascular necrosis (AVN)

Defined: Bones, like other organs in the body, need blood to stay alive and healthy. Unfortunately, sickle red blood cells can sometimes block the blood supply to the round part of either the hip or shoulder bone. This lack of blood flow causes the bone to die, resulting in long-term (chronic) hip or shoulder pain.

How is AVN diagnosed?

The best way to diagnose AVN is through magnetic resonance imaging (MRI). MRI gives a very good picture of the joint without any radiation.

How is AVN treated?

The bone damage in AVN unfortunately cannot be easily fixed. Pain medications and physical therapy can help. If AVN is causing significant problems, an orthopedic surgeon may recommend core decompression surgery or joint replacement surgery.

9. Bone marrow

Bone marrow is the "factory" that produces the different types of blood cells—white blood cells, red blood cells, and platelets.

Aplastic crisis

Defined: Aplastic crisis occurs when the bone marrow "factory" shuts down and stops making blood cells. Some viral infections can cause this aplastic crisis and temporarily stop the production of blood cells. People with sickle cell disease need to constantly make red blood cells since their red blood cells do not typically live as long. When the production of red blood cells stops, the number of red blood cells in the body can drop to a dangerously low level.

10. Blood

Blood transfusions can be life saving for people with sickle cell disease in emergency situations like severe acute chest syndrome. Chronic blood transfusions (scheduled blood transfusions every 3-4 weeks) are also important for some people with sickle cell disease like individuals who have had a stroke. While blood transfusion therapy is very helpful, transfusions also have risks such as alloimmunization and iron overload.

Alloimmunization: The immune system is like a national army protecting your body from foreign invaders. It functions to search and destroy all things in your body that are not you, like bacteria and viruses. One weapon the immune system makes to attack pathogens is antibodies. Antibodies are like little missiles that attach to foreign particles and mark them as an enemy to be destroyed.

What does the immune system have to do with blood transfusions?

The immune system can sometimes identify life saving donor cells as "foreign" since your body did not make them. When this process happens your body makes antibodies against the donor cells. For example, when an individual has made antibodies against a particular red blood cell, if he or she receives a blood transfusion of that type of red blood cell, it would be destroyed and could cause major problems. These antibodies make it more difficult to obtain a blood donor match.

Just like it is crucial for you to know your allergies to medications, it is very important to know if you have developed any antibodies to donor red blood cells. This information can help find the best blood for you in the blood bank next time you need a transfusion. Information about alloantibodies to red blood cells should be on a medical alert bracelet that can "speak for you" when you are in an emergency situation.

Iron overload: Iron is an important mineral used by the body, however, too much iron causes problems. Blood contains iron so when an individual receives a blood transfusion, he or she also receives a significant amount of iron. The body does not remove iron well, so over time if an individual has had many blood transfusions, the amount of iron in his or her body can rise to dangerous levels. In particular, iron can cause problems in the heart, liver, and endocrine glands.

How do you get rid of iron?

If your iron level reaches a certain level, you should start taking a medication to remove iron called "ExJade". It is very important to take this medication every day to bring the iron in your body to a safe level.

Pain

Pain

Staying Healthy

It is very important to remember that there are things that you can do to try to stay healthy and avoid pain crisis.

  • Drink at least 2-3 liters (70-100 oz) of water daily (equals about 4-6 bottles of water)
  • Eat a well balanced diet
  • Don't smoke, drink alcohol, or use street drugs
  • Sleep 8-9 hours every night
  • Avoid very hot or cold weather
  • Go to your doctor for regular check-ups
  • Take your prescribed medicines every day
  • Get your influenza (flu) vaccine every fall

You also need to know your limits. People with sickle cell disease should avoid certain activities. However, remember the list of things that you can do is much longer! If you have any questions about a specific activity ask your doctor. You know your body best, so with experience, you will find what activities are best for you.

Hydroxyurea

Hydroxyurea is currently the only approved drug to treat sickle cell disease. Hydroxyurea has helped many people living with sickle cell disease.

Not all people with sickle cell disease should take hydroxyurea, but all people with sickle cell disease should know these facts about hydroxyurea:

  • Hydroxyurea has been proven to decrease pain crisis and other complications in both children and adults with sickle cell disease
  • Hydroxyurea is a safe medication when given by experienced doctors and with monitoring of blood counts
  • Hydroxyurea must be taken everyday to work

Talk to your doctor about hydroxyurea and if it is right for you.

Check out how hydroxyurea can change your red blood cells! These three pictures were taken at different times using a microscope looking at the blood of a person with sickle cell disease.

This change in your blood takes time and you need to take your hydroxyurea every day for it to occur.

Dealing with Pain

Relaxation techniques can help when pain hits you. Try these techniques:

1. Think of a calm and happy place--an empty beach, a bright blue sky with clouds, or a mountain sunset. Think of details in this place--sounds you enjoy, things you like to touch, taste, and smell.

2. Deep breathing. As you inhale, think about fresh air coming slowly intro your chest, out to your fingers and down to your toes. As you breathe out, imagine that any tension that may have been stored in your arms, neck or shoulders is moving from your body and out into space.

3. Muscle exercises. Get in a comfortable position in a relaxing place. Tighten the different muscles in your body and then relax them, allowing them to go limp. Start with making a fist and then releasing your fingers. Slowly move on to your arms, shoulders, neck, face muscles, toes, legs, and stomach.

Stress. Stress can trigger pain and make existing pain even worse. Learn how to mange your stress. Figure out what works for you.

Support system. A support system is a network of people who help you when you are going through difficult times. Individuals can have different groups of people as a support system. Having a group of people who you can turn to for help is critical. Build up your support system.

Pain medications

There are many different pain medications. You will find that certain medications may work better for your pain. You should know these basic facts about different pain meds:

Acetaminophen (trade name Tylenol). You can buy it over the counter (OTC). It can reduce fever. An adult should never take more than 4000 mg of acetaminophen (eight 500 mg pills) in a 24 hour period because too much acetaminophen can cause serious liver problems. Some medications (Tylenol#3, Percocet, Vicodin) combine acetaminophen with another drug. Make sure you read how much acetaminophen your medications contain to ensure that you are not taking too much.

Ibuprofen (trade names Motrin, Advil). You can buy it over the counter (OTC). It is a type of medicine called a nonsteroidal anti-inflammatory drug (NSAID). It can reduce fever. Side effects: can cause stomach upset and ulcers; can increase bleeding. Do not take if you have ulcer, bleeding, or kidney problems. Another NSAID, Ketorolac (trade name Toradol) is often given via IV when someone with sickle cell disease has crisis.

Opiate medicine (also called narcotics). There are many different opiate medications including codeine, hydrocodone, oxycodone, morphine, and dilaudid. You need a prescription for these drugs. Side effects: constipation, itching, nausea, drowsiness/impaired thinking. When you start taking an opiate for new pain, you should also take a medicine to prevent constipation (like Miralax). If you take opiates on a regular basis you will develop tolerance.

What is tolerance?

Tolerance is a term used to describe a process that occurs when the body becomes less responsive to a drug after repeated exposure. For example, a person who has never received morphine will likely obtain good relief of pain with 2 mg of morphine. However, a person who has previously received morphine many times may need 8 mg of morphine to obtain the same pain relief. The body becomes used to opiates if they are given over time and higher amounts of the drug are needed to achieve the same effect. The body can even become physically dependent on opiates.

What is the difference between physical dependence and addiction?

Physical dependence occurs when the body becomes so used to receiving narcotics over time that if a person stops taking narcotics, he or she will become sick--have withdrawal symptoms. A person who is physically dependent on a narcotic is not necessarily addicted to narcotics. Addiction refers to a compulsive desire to use a drug despite negative consequences. A person addicted to opiates might lie or steal to obtain opiates and continues to use opiates at the expense of family, friends, and their health. A person who is taking opiates to treat pain is not an addict. While many people with sickle cell disease develop some tolerance to opiates, most people with sickle cell disease do not become addicted to opiates.

Sexual Health

Sexual Health

Puberty and sickle cell disease

Puberty is the word used to describe the physical changes that occur as a child's body becomes an adult body capable of reproduction (having babies). Read more about puberty on kidshealth.org.

How does having sickle cell affect puberty?

People with sickle cell go through all the stages of puberty like people without sickle cell. Usually, the age you start puberty is similar to others in your family. However, people with sickle cell often go through puberty at an older age.

It can be tough not being as tall or as developed as other teenagers your age. Many people (not just people with sickle cell) go through puberty at an older age. You should not feel bad about yourself because your body is changing more slowly than others. It is normal to feel awkward about your body at times, but you should not constantly worry about it. If you are concerned about your pubertal development, talk to your doctor.

Menstrual Cycle (Period)

For example, one study found that girls with SS sickle cell had their first menstrual period 2.4 years later than girls without sickle cell, and girls with SC sickle cell had their period 0.5 years later. Today in the U.S., the average girl has her first period around age 12.5 years. Therefore, on average, a girl with SS sickle cell has her first period around her 15th birthday. These ages are averages, and many girls have their first period over a year before or after these average ages and are still completely normal.

Pregnancy and Sickle Cell Disease

Can I get pregnant if I have sickle cell disease?

Yes. Women with sickle cell can become pregnant. It is important for all women to get medical care during pregnancy (called prenatal care), but it is especially important for pregnant women with sickle cell to be followed closely by a high-risk OB/GYN doctor and sickle cell hematologist. Some pregnant women with sickle cell experience more problems.

Can I have healthy children if I have sickle cell disease?

Yes. While women with sickle cell are more likely to have babies that are born early (pre-term) and that weigh less than average, most children are healthy. However, your child could also have sickle cell disease. See the genetics section to learn more.

When is the best time for me to get pregnant if I have sickle cell disease?

This answer will be different for everyone. The best time will depend on many things including your age, your personal health, your goals in life, and your support system. However, it is best to carefully plan when you want to try to get pregnant after talking with your family and doctor. Unexpected or surprise pregnancies can be difficult for anyone but especially for someone with sickle cell. If you are having sex, it is strongly recommended that you use birth control until you are ready to have a baby.

Birth Control and Sickle Cell Disease

What is birth control?

Birth control (also called contraception) is a medicine, device, or practice that reduces the chance of pregnancy. Birth control medicine contains one or two hormones that affect the normal menstrual cycle to prevent pregnancy.

See the links below for more information on birth control:

What form of birth control should I use?

All people who do not want to get pregnant should use condoms. Condoms are the only birth control method that also protect against sexually transmitted infections.

In general, all women who do not want to get pregnant should use another birth control method in addition to condoms since condoms can fail.

Depot medroxyprogesterone acetate (DMPA), also called "the shot," is the best studied form of birth control for women with sickle cell disease. DepoProvera is an injection containing only one hormone, a type of progesterone (no estrogen), that you receive every three months at your doctor's office. In addition to preventing pregnancy, DepoProvera seems to have additional benefits just for women with sickle cell. Women with sickle cell disease who took DepoProvera had less pain crisis episodes than women who did not take DepoProvera.

Most women with sickle cell can usually safely use most forms of birth control. However, some birth control methods, including most oral contraceptive pills, contain the hormone estrogen, which can increase the risk of blood clots and stroke. Limited formal research has been done to know for sure if birth control containing estrogen is safe for women with sickle cell disease. Despite this lack of research, women with sickle cell disease have taken birth control containing estrogen without experiencing problems.

You should talk more to your doctor about which birth control method is right for you.

I am not on birth control and had sex yesterday without a condom. Is there anything I can do to prevent getting pregnant?

Yes. Emergency contraception (also called Plan B) is a progesterone-only hormone pill that a woman can take after unprotected sex to decrease the chance that she will get pregnant. Women must take the emergency contraception pill within 72 hours after unprotected sex to prevent pregnancy (the sooner you can take it, the better). Women with sickle cell can safely take emergency contraception.

Read more about Plan B:

Priapism

What is priapism?

Priapism is an unwanted, prolonged erection of the penis. It can last for hours and often causes pain. Priapism does not occur because of sexual feelings or desires. Rather it occurs when red blood cells sickle and change the chemistry of the blood, causing a blockage of normal blood flow draining from the penis.

Why should I care about priapism?

An erection that lasts over four hours can cause permanent damage to the penis. If untreated, priapism can cause impotence, which means that a man is unable to have an erection for sex.

What should I do if I have an erection that won't go down?

If you have a persistent erection, you should:

  1. Try to urinate.
  2. Drink plenty of fluids.
  3. Take pain medicine.

If the erection lasts longer than one hour then you need to immediately go to the emergency room. At the ED, you should receive IV fluids and pain medicine. If the erection remains, then an urologist (a surgeon who specializes in the penis) may need to give you an injection.

It is very important to go to the hospital if you have priapism. Do not ignore it or try to just deal with it at home. If you have priapism and wait more than two hours to go to the ED, you could cause irreversible and severe damage to your penis. Priapism is serious and not your fault; do not feel embarrassed to get help for it.

Does priapism follow a pattern?

It often occurs as a severe long episode requiring hospitalization and follows multiple shorter episodes, termed “stuttering.” Episodes most often come from infection or normal night-time erections. Onset in the early morning, awakening the patient, is common.

Is it safe for me to masturbate?

Generally, priapism is not related to sexual activity. Rather, it often occurs during sleep. However, priapism can sometimes occur after sex. Despite this fact, masturbation is safe and usually does not cause priapism. If you have any concerns about having sex or masturbation, talk to your doctor.

Will I be able to father children?

Men with sickle cell disease are more likely to have problems with fertility. Men with sickle cell disease may have a more difficult time getting a woman pregnant because sickle cell disease can affect sperm. However, men with sickle cell can get a woman pregnant and father children. If you have concerns about your ability to father a child, talk to your doctor.

Genetic Counseling and Sickle Cell Disease

If I have a child what are the chances that my child will also have sickle cell disease?

The chances of having a child with sickle cell disease, if you have sickle cell disease, depends on the type of sickle cell disease that you have (SS, SC, Sβthal) and your partner's hemoglobin genes. Since there are different types of sickle cell disease and hemoglobin, it is best for a genetic counselor or doctor to personally explain your specific chances of having a child with sickle cell disease based on you and your partner's hemoglobin types.

In general, these are the guidelines for a person with sickle cell disease:

  • If your partner also has sickle cell disease, then there is close to a 100 percent chance that your child will have sickle cell disease.
  • If your partner has a form of sickle cell trait, then there is a 50 percent chance that your child will have sickle cell disease.
  • If your partner does not have sickle cell disease or trait, then there is a close to zero percent chance that your child will have sickle cell disease.

I have sickle cell disease (SS) and my partner has trait (AS). We have already had one child with sickle cell disease (SS). If we have another child, is he/she more likely or less likely to have sickle cell disease?

Remember that the chance that your child will have sickle cell disease is independent for each pregnancy—meaning that it does not matter if your previous child did or did not have sickle cell disease. The chance that the above person would have a child with sickle cell disease is 50 percent.

What should I do if there is a chance my child could have sickle cell disease?

Some people may tell you not to have children. Others may tell you to only have children with someone who doesn't have trait. But what if you fall in love with someone who does have trait? There are many important features that you look for in a partner, and whether or not they have sickle trait may not rank high on your list, and it certainly doesn't have to. These are tough questions and issues, and there is no right answer. You need to think about the chances and discuss it with your partner before you have children.

Most importantly, you should choose to have children with someone who loves you, understands you, and plans to be with you for a lifetime. Having children is a huge responsibility, and you want to make sure that you and your partner are in a position to provide for your children's needs, which includes having a stable and loving home. Once you are ready, you should meet with a genetic counselor or doctor. He or she can provide more information about your specific chance of having a child with sickle cell disease and the options you have to deal with that chance. There is no one correct choice; the answer is different for each couple, and each couple makes decisions for different reasons. It is important that you take the time to consider the options, and make the choice that is right for you and your partner.

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