Transitioning Your Medical Care: Sickle Cell Disease
If you have sickle cell disease, a pediatric (childhood) 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. Now is a good time to start looking into "transitioning" from your pediatric specialist to doctors who treat adults.
What Does it Mean to Transition Health Care?
When doctors talk about "transition of care," they mean safely shifting a teen's health care from a pediatric to an adult medical practice. But who takes care of you is just one part of this. Transitioning also means learning how to take charge of your own health and manage every aspect of your care, from handling insurance forms to understanding and making decisions about treatments.
Your parents have probably played a big part in your medical care, and that's great. But after you turn 18, they may not be able to see your medical records or talk about your health with your doctor — even if you want them to.
Your medical team should be able to guide you in the transitioning process, but here are some good things to know.
How Can I Prepare to Transition to Adult Care?
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 take charge and have more responsibility.
It helps to get ready by being more involved in your health care right now now. Experts recommend that teens take steps to learn about their medical care around age 13 or 14. Here's where to start:
- Keep track of your appointments.
- When you arrive at the doctor's office, check in at the window yourself (instead of relying on a parent or caregiver to do it).
- Keep a list of your current medications and update it when there are changes.
- Refill your own prescriptions.
- Know your medical information, like any food and drug allergies you may have.
When Should Teens With Sickle Cell Disease Transition to Adult Care?
Most people have to go to a medical practice for adults somewhere between turning 18 and 21. Often, your children's hospital or doctor's office will have a policy on the age at which they can no longer see patients. This cutoff age might be so fixed that you can make an appointment with your current doctor for one day but not be allowed to make one on the next day. The best time to start seeing adult doctors is well before you hit this cutoff age.
Ask your doctor to recommend an adult hematologist (and any other doctors you need to see) and make appointments with these new doctors at least a year before the date your current doctor will have to stop seeing you. That way, if you have questions or if something doesn't feel quite right, there is time to go back to your pediatric doctor for help making your transition.
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 all the medical information. But they will assume that you know what to do when it comes to living with sickle cell disease.
For instance, your adult hematologist will expect you to know:
- what to do when you're running a temperature
- what medicines to try for mild and moderate pain at home
- how to stay well hydrated
- when to go to the office, a sickle cell treatment center, or the ER
Each patient with sickle cell disease is a little different, so discuss these topics 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. Most of the time, adult 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 (if you have asthma, you might need PFTs more often)
- get screening labs and urine studies every year
- possibly need imaging studies done like X-rays, MRIs, or ultrasounds
Every person with sickle cell disease has a unique life story. Some people have fairly mild disease with occasional pain crises. Others may spend lots of days in the hospital.
Most of sickle cell disease do not change from adolescence to adulthood. But it's good to be aware of what can happen.
As you get older, you may find your pain episodes change. For example, you might have less intense belly pain but more long-lasting bone or muscle pain. The way doctors treat your pain will also be different. Because of the risk of side effects from some pain medicines, doctors often expect adults to manage pain using heat or relaxation techniques in addition to taking medicine.
If your sickle cell pain often takes you to the ER and you can no longer go to a children's hospital, try to build a relationship with a new emergency room. Some doctors recommend you work with your hematology team and hospital emergency room to create a pain plan. Using a pain plan can help the ER better treat your sickle cell pain.
Find out if there is a day treatment center for sickle cell disease in your area. These centers are in many larger cities. They have special training in sickle cell pain and might be able to help control your pain faster than a regular emergency room.
Leg ulcers may become more common in adulthood. If you start getting leg ulcers, you may need creams and even antibiotics. Good skin care and avoiding skin damage can help to prevent ulcers.
Sickle cell disease can make getting pregnant more difficult. Women with sickle cell disease can still get pregnant, though. So if you're not ready to have a baby, you still need to use birth control.
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, women with sickle cell disease need to 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 might prescribe medicine to help prevent priapism.
Bedwetting is extremely common with sickle cell disease, but it's not caused by the extra water people drink. In fact, it's the opposite: Over time, sickle cell disease can cause blood in the kidneys to get thicker and not flow as well. The blood might not get to parts of the kidney, so the kidneys get damaged. Since drinking water is a way to help manage sickling, you'll want to drink as much as you can.
People who wet the bed tend to do it at the same time each night. Setting a timer or cellphone 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. If your spleen has been removed, you should also get a pneumococcal vaccine.
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 that 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 dorm.
Doctors recommend that everyone get the flu vaccine each year. A flu shot is very important for people with sickle cell because they have a higher chance of problems if they get the flu. You'll need to get the flu shot (which uses killed flu viruses), not the nasal spray (which has live viruses in it).
Where to Find Info 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 can point you in the right direction.
Maybe a member of the hematology team can 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 if 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 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.