Hereditary Spherocytosis in My Baby
Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist
Content reviewed against published ASH, AAP, NIH guidelines
Last reviewed:
If your baby has been diagnosed with or you suspect hereditary spherocytosis in my baby, here is what the evidence says.
The short answer
Hereditary spherocytosis (HS) is the most common inherited hemolytic anemia in people of northern European descent. Red blood cells are sphere-shaped instead of disc-shaped, causing them to be trapped and destroyed in the spleen. This leads to anemia, jaundice (yellowing of skin and eyes), and an enlarged spleen. Severity ranges from mild (compensated) to severe. In newborns, it often presents as prolonged or severe jaundice. Most children with HS lead normal lives with monitoring, and some require splenectomy later in childhood.
Key takeaways
- Hereditary spherocytosis (HS) is the most common inherited hemolytic anemia in people of northern European descent. Red blood cells are sphere-shaped instead of disc-shaped, causing them to be trapped and destroyed in the spleen. This leads to anemia, jaundice (yellowing of skin and eyes), and an enlarged spleen. Severity ranges from mild (compensated) to severe. In newborns, it often presents as prolonged or severe jaundice. Most children with HS lead normal lives with monitoring, and some require splenectomy later in childhood.
- Usually normal when: Mild jaundice in a newborn that resolves with standard phototherapy and does not recur
- Call your doctor if: Your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion
- Varies by age — see the age-by-age breakdown below
“Fever itself is not an illness — rather, it is a sign or symptom that the body is fighting an infection. Fever stimulates certain defenses, such as the white blood cells, which attack and destroy invading bacteria.”
Parents everywhere have the same worry. You are doing the right thing by looking into it.
What Parents Should Know
According to ASH, AAP, NIH guidelines, hereditary spherocytosis (HS) is the most common inherited hemolytic anemia in people of northern European descent. Red blood cells are sphere-shaped instead of disc-shaped, causing them to be trapped and destroyed in the spleen. This leads to anemia, jaundice (yellowing of skin and eyes), and an enlarged spleen. Severity ranges from mild (compensated) to severe. In newborns, it often presents as prolonged or severe jaundice. Most children with HS lead normal lives with monitoring, and some require splenectomy later in childhood. At 0-2 weeks, hereditary spherocytosis often first presents in newborns as significant or prolonged jaundice that may require phototherapy or, in severe cases, exchange transfusion. The jaundice occurs because sphere-shaped red blood cells are destroyed more rapidly, releasing bilirubin. A direct Coombs test is negative (distinguishing HS from immune-mediated hemolysis). The blood smear shows spherocytes (small, round red blood cells without the normal central pallor). A family history of HS, anemia, jaundice, gallstones, or splenectomy in a parent is a strong clue. It is generally considered normal when mild jaundice in a newborn that resolves with standard phototherapy and does not recur. However, you should contact your pediatrician promptly if your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion.
Normal vs. Concerning
When to Seek Immediate Care
- Your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion
- Your child with HS develops high fever and appears very ill, especially if they have had a splenectomy, as this could be overwhelming post-splenectomy sepsis requiring immediate antibiotics and emergency care
- A newborn has severe jaundice with bilirubin levels rising rapidly despite phototherapy, which may require exchange transfusion
By Age
What to expect by age
0-2 weeks
Hereditary spherocytosis often first presents in newborns as significant or prolonged jaundice that may require phototherapy or, in severe cases, exchange transfusion. The jaundice occurs because sphere-shaped red blood cells are destroyed more rapidly, releasing bilirubin. A direct Coombs test is negative (distinguishing HS from immune-mediated hemolysis). The blood smear shows spherocytes (small, round red blood cells without the normal central pallor). A family history of HS, anemia, jaundice, gallstones, or splenectomy in a parent is a strong clue.
2 weeks - 3 months
Infants with HS may develop worsening anemia during this period as their normal physiologic anemia of infancy is compounded by ongoing hemolysis. Some babies require one or more blood transfusions in the first few months of life, particularly those with severe HS. Reticulocyte counts (young red blood cells) will be elevated as the bone marrow tries to compensate for the red blood cell destruction. Folic acid supplementation is typically recommended to support increased red blood cell production.
3-12 months
By this age, the severity of HS becomes more apparent. Children with mild HS may have compensated hemolysis (the bone marrow keeps up with red blood cell destruction) and require no transfusions. Those with moderate to severe HS may need occasional transfusions, especially during viral infections (aplastic crises caused by parvovirus B19) when the bone marrow temporarily stops producing red blood cells. The spleen may become palpably enlarged. Diagnosis is confirmed with an osmotic fragility test or eosin-5-maleimide (EMA) binding test.
1-5 years
During early childhood, the main concerns are managing anemia, monitoring for aplastic crises during infections, and watching for gallstones (which can develop from chronic hemolysis even in young children). Splenectomy is considered for children with severe HS requiring frequent transfusions, but it is generally deferred until after age 5 when possible to reduce the risk of overwhelming post-splenectomy infection. Partial splenectomy may be considered in younger children. All children with HS should receive pneumococcal and meningococcal vaccines, especially before any planned splenectomy.
What to Tell Your Pediatrician
- Describe when you first noticed hereditary spherocytosis in my baby and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if your baby's jaundice is lasting longer than expected or recurs after initial treatment.
- Mention if your child with known HS seems more tired, pale, or irritable than usual, which may indicate worsening anemia.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
What Should You Do?
When to take action
- Mild jaundice in a newborn that resolves with standard phototherapy and does not recur
- A family member has known mild HS with compensated hemolysis requiring no treatment
- Your child has mild HS with a stable hemoglobin above 9-10 g/dL, mild reticulocytosis, and no symptoms
- Slight splenic enlargement that remains stable and your child is active and growing well
- Your baby's jaundice is lasting longer than expected or recurs after initial treatment
- Your child with known HS seems more tired, pale, or irritable than usual, which may indicate worsening anemia
- You notice your child's abdomen seems swollen or you can feel an enlarged spleen
- Your child develops abdominal pain that could indicate gallstones from chronic hemolysis
- Your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion
- Your child with HS develops high fever and appears very ill, especially if they have had a splenectomy, as this could be overwhelming post-splenectomy sepsis requiring immediate antibiotics and emergency care
- A newborn has severe jaundice with bilirubin levels rising rapidly despite phototherapy, which may require exchange transfusion
What You Can Do at Home
- Keep track of when you notice hereditary spherocytosis in my baby — noting the time of day, duration, and any triggers can help your pediatrician.
- Remember that mild jaundice in a newborn that resolves with standard phototherapy and does not recur — this is generally within the range of normal.
- At 0-2 weeks, focus on observation rather than intervention unless your pediatrician advises otherwise.
- Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
- While monitoring at home, seek immediate care if your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion.
Related Conditions
My Baby Looks Very Pale
Babies' skin color can vary naturally depending on temperature, activity, and genetics. However, true pallor (unusual paleness of the skin, lips, or nail beds) can sometimes indicate anemia, poor circulation, or an infection. If your baby looks noticeably paler than usual, especially in the lips, gums, inner eyelids, or palms, it is worth mentioning to your pediatrician.
Iron Deficiency Anemia in Babies
Iron deficiency is the most common nutritional deficiency in young children. Babies are born with iron stores that last about 4-6 months, after which they need dietary iron from breast milk (with supplementation), formula, or iron-rich foods. Untreated iron deficiency can affect brain development and cognitive function, so early detection and treatment are important.
Thalassemia in My Baby - Trait vs Disease
Thalassemia is a group of inherited blood disorders where the body makes abnormal or insufficient hemoglobin, leading to anemia. There are two main types: alpha thalassemia and beta thalassemia. Thalassemia trait (minor) causes mild or no anemia and usually requires no treatment. Thalassemia disease (major or intermedia) causes more significant anemia that may require regular blood transfusions. Most cases are identified through newborn screening. Families with Mediterranean, Middle Eastern, African, or Southeast Asian heritage are at higher risk.
My Baby Has G6PD Deficiency — What Do I Need to Avoid?
G6PD (glucose-6-phosphate dehydrogenase) deficiency is the most common enzyme deficiency worldwide, affecting approximately 400 million people. It is an X-linked condition, meaning it primarily affects males. G6PD helps protect red blood cells from damage. When triggered by certain foods, medications, infections, or chemicals, a baby with G6PD deficiency can have a hemolytic episode — their red blood cells break down faster than the body can replace them, causing anemia and jaundice. The key to managing G6PD deficiency is knowing and avoiding triggers. Between episodes, children with G6PD deficiency are completely healthy.
Related Resources
Jaundice Decision Tree
Assess jaundice severity in newborns and when to call the doctor.
Month-by-Month Development
Detailed monthly development guides from birth through 24 months.
When to Call the Doctor
General guide on when to call the pediatrician, visit urgent care, or go to the ER.
Frequently asked questions
Is hereditary spherocytosis in my baby normal?
When should I call the doctor about hereditary spherocytosis in my baby?
When is hereditary spherocytosis in my baby normal?
What causes hereditary spherocytosis in my baby?
What should I mention to my pediatrician about hereditary spherocytosis in my baby?
Is hereditary spherocytosis in my baby normal at 0-2 weeks?
Is hereditary spherocytosis in my baby normal at 2 weeks - 3 months?
Should I go to the ER for hereditary spherocytosis in my baby?
Does hereditary spherocytosis in my baby go away on its own?
References
- [1]Bolton-Maggs PHB, Langer JC, Iolascon A, Tittensor P, King MJ. Guidelines for the Diagnosis and Management of Hereditary Spherocytosis - 2011 Update. British Journal of Haematology. 2012;156(1):37-49. ASH
- [2]American Academy of Pediatrics. Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation. Pediatrics. 2004;114(1):297-316. AAP
- [3]National Organization for Rare Disorders (NORD). Hereditary Spherocytosis. NIH
Doctor Visit Checklist
Bring this checklist to your next pediatrician visit to discuss Hereditary Spherocytosis in My Baby.
Things to mention
- Describe when you first noticed hereditary spherocytosis in my baby and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if your baby's jaundice is lasting longer than expected or recurs after initial treatment.
- Mention if your child with known HS seems more tired, pale, or irritable than usual, which may indicate worsening anemia.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
Observations to share
- Your baby's jaundice is lasting longer than expected or recurs after initial treatment
- Your child with known HS seems more tired, pale, or irritable than usual, which may indicate worsening anemia
- You notice your child's abdomen seems swollen or you can feel an enlarged spleen
Urgent signs to report immediately
- Your baby or child with HS suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus B19 infection) requiring emergency transfusion
- Your child with HS develops high fever and appears very ill, especially if they have had a splenectomy, as this could be overwhelming post-splenectomy sepsis requiring immediate antibiotics and emergency care
- A newborn has severe jaundice with bilirubin levels rising rapidly despite phototherapy, which may require exchange transfusion
My notes
From ismybabyalright.com — free, evidence-based baby health guides
All content follows our editorial policy and is reviewed against published clinical guidelines.
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Related Resources
Bottom line
Most cases of hereditary spherocytosis in my baby are normal. Talk to your pediatrician if your baby or child with hs suddenly becomes very pale, lethargic, and has a rapid heartbeat, which could indicate an aplastic crisis (often triggered by parvovirus b19 infection) requiring emergency transfusion.
Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.
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Related Medical Concerns
My Baby Looks Very Pale
Babies' skin color can vary naturally depending on temperature, activity, and genetics. However, true pallor (unusual paleness of the skin, lips, or nail beds) can sometimes indicate anemia, poor circulation, or an infection. If your baby looks noticeably paler than usual, especially in the lips, gums, inner eyelids, or palms, it is worth mentioning to your pediatrician.
Iron Deficiency Anemia in Babies
Iron deficiency is the most common nutritional deficiency in young children. Babies are born with iron stores that last about 4-6 months, after which they need dietary iron from breast milk (with supplementation), formula, or iron-rich foods. Untreated iron deficiency can affect brain development and cognitive function, so early detection and treatment are important.
Thalassemia in My Baby - Trait vs Disease
Thalassemia is a group of inherited blood disorders where the body makes abnormal or insufficient hemoglobin, leading to anemia. There are two main types: alpha thalassemia and beta thalassemia. Thalassemia trait (minor) causes mild or no anemia and usually requires no treatment. Thalassemia disease (major or intermedia) causes more significant anemia that may require regular blood transfusions. Most cases are identified through newborn screening. Families with Mediterranean, Middle Eastern, African, or Southeast Asian heritage are at higher risk.
My Baby Has G6PD Deficiency — What Do I Need to Avoid?
G6PD (glucose-6-phosphate dehydrogenase) deficiency is the most common enzyme deficiency worldwide, affecting approximately 400 million people. It is an X-linked condition, meaning it primarily affects males. G6PD helps protect red blood cells from damage. When triggered by certain foods, medications, infections, or chemicals, a baby with G6PD deficiency can have a hemolytic episode — their red blood cells break down faster than the body can replace them, causing anemia and jaundice. The key to managing G6PD deficiency is knowing and avoiding triggers. Between episodes, children with G6PD deficiency are completely healthy.
Physiologic Anemia of Infancy - Normal Hemoglobin Drop
Physiologic anemia of infancy is a normal, expected drop in hemoglobin levels that occurs in all babies around 6 to 8 weeks of age. After birth, the baby transitions from fetal hemoglobin to adult hemoglobin, and red blood cell production temporarily decreases as the body adjusts to the oxygen-rich environment outside the womb. In full-term babies, hemoglobin typically drops to about 9.5-11 g/dL and then recovers on its own without treatment. In premature babies, the drop can be more pronounced (anemia of prematurity) and may occasionally require intervention.
My Baby's Head Shape Looks Abnormal
Many babies develop temporary head shape irregularities that are completely normal. A cone-shaped head from vaginal delivery reshapes within days. Mild positional flattening (plagiocephaly) from sleeping on the back is very common and usually improves with repositioning and tummy time. However, head shape changes involving ridges, a persistently bulging fontanelle, or rapid head growth changes should be evaluated to rule out craniosynostosis.