Transitioning Your Medical Care: Sickle Cell Disease
If you were born with sickle cell disease, a pediatric (childhood) hematologist is probably directing your medical care. That doctor and the hematology team have been there to support you and your family. They’ve been there if you had a fever and needed to stay in the hospital or had pain that was too severe to be cared for at home.
Sickle cell disease will be with you throughout your life, in some way or another. As you grow older, you will have questions about how it may affect you in the future. That’s a good time to start looking into “transitioning” from your pediatric specialist to doctors who treat adults.
What Is Transitioning?
When doctors talk about “transitioning,” they mean safely shifting a patient’s medical care from a pediatric to an adult-oriented practice. Your new medical “home” could be a sickle cell care center or the office of an adult hematologist.
The key word is safely. You’ll need to take lots of steps before you can make a smooth switch to adult health care. Your medical team should be able to guide you in the transitioning process, but here are some good things to know.
How to Prepare
Adult care centers are very different from pediatric offices. The biggest change is often the amount of input and responsibility you’ll have. As an adult, you’re expected to be more involved in making decisions.
It helps to get ready to play a bigger role by being more involved in your health care as a teenager. You can start by:
- Keeping track of appointments.
- Checking in at the window yourself (instead of relying on a parent or caregiver to do it).
- Keeping lists of your current medications.
- Refilling your own prescriptions.
- Knowing medical information, like any food and drug allergies you may have.
When to Switch Doctors
Somewhere between ages 18 and 21 most patients will need to start going to a medical practice for adults. Often, pediatric doctors are unable to see patients older than a certain age because of an office or hospital policy.
The best time to start seeing an adult hematologist is before you get too old to see the pediatric doctor. This way, if you have questions or if something doesn’t feel quite right, there is time to return to the pediatric hematologist and discuss the transition more.
What to Expect From Your New Hematologist
Adult hematologists expect their patients to know more about sickle cell disease than you’re probably used to. Obviously they won’t require you to know the medical information, but they will assume that you have practical knowledge about living with sickle cell disease.
For instance, your adult hematologist will expect you to:
- know what to do when you’re running a temperature
- know what medications to try for mild and moderate pain at home
- know how to stay well hydrated
- know when to go to the office, a sickle cell treatment center, or the ER
Because each patient with sickle cell disease is a little different, discuss these questions with your pediatric hematologist before you move on to adult care.
As you meet with your new adult hematologist you will need to discuss your bloodwork schedule and other routine testing. Typically, patients with sickle cell disease will:
- see the hematologist every 6 to 12 months
- see an eye doctor every year
- need pulmonary function tests (PFT) once as a teenager and once as a young adult. Additional PFTs may be needed in patients who have asthma.
- get screening labs and urine studies every year
- possibly need imaging studies done like X-rays or ultrasounds
Every person with sickle cell disease has a unique life story. Some people have relatively mild disease with only the occasional pain crises. Others may have spent multiple days in the hospital with complications of sickle cell disease.
Most of the symptoms of sickle cell disease do not change from adolescence to adulthood. But it’s good to be aware of possibilities, including things like how to manage pain and sexual health care.
One of the well-known symptoms of sickle cell disease is pain (or vaso-occlusive crisis). Pain episodes may change a little with age. For example, young children with sickle cell pain may have belly pain as their most severe pain. In older children and adults, bone and muscle pain is more likely. Adults may not see painful swollen fingers or toes (dactylitis), but arthritis in fingers and other joints may continue through adulthood.
You’ll still need to get treatment for pain episodes. But the way pain is treated might seem a little different. As a child, you most likely went to the same pediatric hospital every time you had sickle cell pain. Nurses and ER doctors probably knew you and were familiar with your pain needs and your sickle cell history. Your hematologist may have been on staff at that hospital.
In the adult world, you might need to re-create this relationship with a new emergency room. Some doctors recommend establishing a pain plan with the adult hospital emergency room and your hematology team. Using a pain plan can help the emergency room better treat your sickle cell pain.
If possible, find out if there is a day treatment center for sickle cell disease near where you will be living. This type of health center is trained specifically to help patients with sickle cell pain. Because of their expertise, they may be able to help control your pain faster than a regular emergency room.
The more often you take certain pain medications, the higher a dose you will need to have that specific medication control your pain. Unfortunately, all medications have side effects and people generally have more side effects at higher doses, even if the medicine is barely enough to control the pain. Because of this, doctors generally like to give small amounts of medication to try and avoid side effects.
Many pain medicines will start to work within 30 minutes. So if you are not feeling better within half an hour after getting the medication, let your doctor know. Again, having a pain plan — and letting doctors know what has worked for you in the past — will help in treating your pain.
Children and teens rarely develop leg ulcers from sickle cell disease. Leg ulcers may become more common in adulthood, though. If they develop, you may need creams and even oral antibiotics. Good skin care and avoiding skin damage can help to prevent ulcers.
Sickle cell disease will not prevent a woman from getting pregnant, although it may make getting pregnant more difficult.
If you are pregnant or plan to get pregnant, talk to your hematologist. Pregnant women with sickle cell disease need good medical care because their babies are at risk for being born early. If you’re pregnant, you’ll want to find an obstetrician (OB) who specializes in high-risk pregnancy.
All pregnant women should take folate to decrease the risk of birth defects. Because folate is used to make red blood cells, patients with sickle cell disease should be on a higher dose of folate during pregnancy.
Sickle cell disease affects sperm development. Men with sickle cell disease can still father a child, but it may be difficult.
One condition that can affect men with sickle cell is priapism (the medical term for a painful erection lasting more than 3-4 hours). Go to the emergency room immediately if you are having a long-lasting, painful erection. It may seem embarrassing, but if priapism isn’t treated, a guy may not be able have an erection in the future.
Some men with sickle cell disease have painful erections that last for 1-2 hours. These can be a warning sign that you are at risk for priapism. Talk to your hematologist, who may be able to prescribe medicine to help prevent priapism.
Bedwetting is extremely common with sickle cell disease, but it’s not caused by drinking too much water before bedtime. In fact, many people with sickle cell disease who have trouble staying dry at night don’t get enough fluids to stay well hydrated. Sickle cell disease makes it difficult over time for the body to reabsorb water in the kidneys, so the body makes lots of urine.
People who wet the bed tend to do it at the same time each night. Setting a timer or cell phone alarm to wake you up so you can use the bathroom may help prevent bedwetting.
Infection and Immunizations
Sickle cell disease affects how people fight off infections. Your doctor might prescribe daily antibiotics to help prevent serious infections. Some people who’ve had their spleens removed need to stay on antibiotics for life.
Even if you’re taking antibiotics, a fever may still be a medical emergency. If you develop a fever, call your hematologist for advice right away.
Doctors recommend all patients with sickle cell disease get a meningococcal vaccination. This is a must for people who are going to have their spleens taken out or who plan to live in a college dormitory.
Doctors recommend that everyone get the flu vaccine each year. A flu shot is especially important for people with sickle cell, who are at higher risk of getting flu complications. The live viruses in the nasal spray can pose a risk for people with sickle cell. So you’ll need to get the shot (which contains killed flu viruses), not the spray. Your doctor can advise you on this.
Where to Find Information About Adult Hematologists
Your pediatric hematologist is probably the best person to ask about adult hematologists. Your current doctor will have lots of previous patients who have graduated to adult care and so can point you in the right direction.
Sometimes, someone from your hematology team might be able to go with you to your first adult appointment. Or perhaps the adult hematologist can join you at your last appointment with your pediatric hematologist. If this is something you’re interested in, talk to the medical team and see how it can be arranged.
What About Other Doctors?
If you’re living with sickle cell, you’ve probably seen a specialist at some point — like an ophthalmologist or pulmonologist. If he or she is a pediatric doctor, you’ll need to transition to an adult specialist. Your current specialist and your hematology teams (both pediatric and adult) can recommend new specialists and help you make the transition.
No matter how well a person prepares, there will always be a few unexpected questions and bumps in the road. That’s OK — transitioning to adult care is a journey, not a single leap. The important thing is to make sure you’re always covered, so start thinking about transitioning when you’re in your mid-teens.
Reviewed by: Cory Ellen Nourie, MSS, MLSP
Date reviewed: August 2011