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Long-Term Complications and Transitioning to Adult Care

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Transitioning from pediatric to adult sickle cell care requires early planning to avoid gaps in treatment. Adults with SCD must monitor for long-term complications from chronic low oxygen, including avascular necrosis of the hip, iron overload from transfusions, gallstones, and silent strokes.

Key Takeaways

  • • The transition from pediatric to adult care is a high-risk period due to insurance changes, provider shortages, and care gaps.
  • • Avascular necrosis frequently affects the hip joint in SCD, requiring early MRI detection and sometimes a total hip replacement.
  • • Frequent blood transfusions can cause toxic iron overload, which is managed with iron chelation therapy.
  • • Silent cerebral infarcts are common brain injuries in SCD that can negatively impact focus and executive function.
  • • Patients should start preparing for adult care in their early teens by learning to manage prescriptions and medical appointments.

As a person with sickle cell disease (SCD) moves from childhood into adulthood, the focus of medical care shifts. While childhood is often marked by acute events like infections, adulthood brings the challenge of managing the cumulative, long-term effects of chronic tissue hypoxia (low oxygen) and recurrent vaso-occlusion (blocked blood flow) [1][2].

Long-Term Physical Complications

Over time, the repeated cycle of red blood cells sickling and unsickling can damage almost any organ in the body.

Avascular Necrosis (AVN)

Avascular Necrosis (AVN) is the death of bone tissue due to a lack of blood supply [3]. In SCD, this most commonly affects the head of the femur (the ball of the hip joint) [4].

  • Signs: The first sign is usually a dull ache or throbbing in the hip or groin area that gets worse with movement [3][5].
  • Management: Early detection via MRI is critical [6]. Many adults eventually require a total hip replacement to regain mobility and reduce pain [7][8].

Iron Overload

Many patients with SCD receive regular blood transfusions to treat severe anemia, prevent strokes, or manage acute chest syndrome. While transfusions are life-saving, blood contains a lot of iron, and the body has no natural way to get rid of the excess [9]. Over time, this iron builds up and can cause toxic damage to the heart and liver. Patients receiving frequent transfusions will likely need iron chelation therapy—a medication that helps the body process and excrete the extra iron [9].

Gallstones (Cholelithiasis)

Because sickle cells break down much faster than normal cells (hemolysis), the body produces a waste product called bilirubin at a very high rate [10][11]. This excess bilirubin can harden into pigment gallstones [10]. If the stones cause pain or blockages, a surgeon may need to remove the gallbladder [10][12].

Leg Ulcers

Chronic low oxygen and inflammation in the skin can lead to leg ulcers, which are painful, slow-healing sores that usually appear near the ankles [13][14]. They are more common in men and those with the most severe forms of the disease [14].

The “Silent” Challenge: Cerebral Infarcts

Many children and adults with SCD experience silent cerebral infarcts (SCIs)—small areas of brain damage that do not cause an obvious “stroke” but can still be seen on an MRI [15][16].

  • Prevalence: By age 18, over one-third of individuals with sickle cell anemia have at least one SCI [17].
  • Impact: These “silent” events can significantly affect executive function, making it harder to stay focused, plan, or solve complex problems [15][18].

Navigating the Transition to Adult Care

The period between ages 18 and 25 is a time of high vulnerability. Unfortunately, the risk of death actually increases during this “transition” phase [19][20].

Why Is Transition Risky?

  • Care Gaps: Moving from a pediatric team (who often coordinate everything) to the adult system (where the patient is expected to manage their own care) can lead to missed appointments [21].
  • Insurance Issues: Many young adults lose their pediatric insurance coverage, making it harder to afford daily medications like Hydroxyurea [22].
  • Provider Shortages: There are fewer adult hematologists who specialize in SCD compared to pediatric ones [2].

Strategies for Success

  1. Start Early: Begin discussing transition with your pediatric team by age 13 or 14 [23].
  2. Learn the Skills: Practice calling in your own refills, making appointments, and explaining your medical history [24][25].
  3. Find Your Team: Before you leave your pediatric clinic, make sure you have a “warm handoff” to an adult hematologist who has a plan for your care [23][26].

The Emotional Toll and Mental Health

Reading about shortened life expectancy and long-term organ damage is frightening. The emotional and mental weight of living with a chronic, painful disease cannot be overstated. Finding a therapist who specializes in chronic illness, or connecting with patient advocacy groups (such as the Sickle Cell Disease Association of America), is just as important as managing your physical symptoms. You do not have to navigate this alone.

Frequently Asked Questions

What is avascular necrosis in sickle cell disease?
Avascular necrosis is bone death caused by a lack of blood supply, commonly affecting the hip joint in people with sickle cell disease. Early signs include a dull ache or throbbing in the groin that worsens with movement.
Why do sickle cell patients get iron overload?
Many patients receive regular blood transfusions to treat severe anemia or prevent strokes. Because blood contains high amounts of iron and the body cannot naturally eliminate the excess, it builds up and requires iron chelation therapy to prevent organ damage.
What are silent cerebral infarcts?
Silent cerebral infarcts are small areas of brain damage caused by blocked blood flow that do not cause obvious stroke symptoms. They can, however, affect executive function, making it harder to focus, plan, or solve complex problems.
Why is transitioning to adult sickle cell care risky?
Moving from pediatric to adult care can lead to missed appointments, changes in health insurance coverage, and challenges finding an adult hematologist who specializes in sickle cell disease. This gap in care increases the risk of serious health complications.
How can I prepare to move to an adult hematologist?
Start preparing early by learning to fill your own prescriptions, making your own appointments, and understanding your medical history. Ask your pediatric team for a warm handoff to a specialized adult provider before you leave their care.

Questions for Your Doctor

  • • Have I ever had a brain MRI to screen for silent cerebral infarcts, and how might that affect my school or work performance?
  • • What is the specific protocol for transitioning my care from this pediatric clinic to an adult hematologist?
  • • Can you recommend an adult hematologist who has experience managing long-term complications like iron overload or AVN?
  • • I’ve been having hip pain; could this be avascular necrosis, and should we get an MRI to check?
  • • My eyes and skin look a bit yellow; could I have gallstones from chronic hemolysis?

Questions for You

  • • Do I know how to fill my own prescriptions and explain my diagnosis to an ER doctor who might not know me?
  • • Have I ever noticed any small sores or skin changes on my ankles that aren't healing?
  • • Who is my 'point person' for my health insurance, and will my coverage change when I turn 18 or 21?
  • • Do I feel confident making my own medical appointments and advocating for my pain management needs?

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This page provides educational information about sickle cell disease complications and care transitions. It is not a substitute for professional medical advice from your hematologist.

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