Head bleed

Head Bleeds and Hemophilia

What you need to know about intracranial hemorrhage symptoms and treatment
Author: Sarah Aldridge

Hogan Lust started walking last month. He’s two and a half years old. For the tow-headed toddler from Tucson, that represents a developmental milestone no specialist could guarantee he would ever achieve. Hogan had a head bleed when he was one week old. His mother, Corby, knew something was wrong when he stopped nursing and started having a seizure. A call to Hogan’s pediatrician sent her and her husband, Daniel, scrambling to get the baby to the nearest major hospital quickly. As first-time parents with no bleeding disorder history, Hogan’s parents were alarmed.

“My new baby was in the hospital in an intensive care unit hooked up to machines I‘d never heard of, with doctors I’d never met and staff telling me he had a bleeding disorder, which meant long term,” says Corby. “Daniel was trying to keep me calm, but he was panicking, too, he later told me.”

A CAT (CT) scan revealed an intracranial hemorrhage, a bleed in the brain. A pediatric neurosurgeon met with the couple to tell them Hogan needed brain surgery. Pressure was building in the brain from the blood leaking into it, preventing oxygen from circulating. The neurosurgeon’s plan was to remove the frontal lobe and part of the parietal lobe.

“It was very serious,” says Corby. “They said the next 24 to 48 hours were going to be very critical, because they weren’t sure Hogan would make it.”

Hogan did make it, but has had some residual problems. At three months old, he was diagnosed with left-sided hemiplegia, a form of cerebral palsy, which has caused developmental delays in motor skills and speech. Hogan wears glasses to correct far-sightedness and leg braces to stabilize his ankles and support his legs up to the knee. He may never have full use of his left hand and fingers.

Through physical and occupational therapies, however, Hogan has come a long way. He’s regained shoulder control on his left side, can use his left hand as a helper and to give high-fives, and has 50 words in his vocabulary. “The day he gave us high-fives with his left hand was a big day,” says Corby. “My father took us to the local watering hole to celebrate.”

Still, the family knows the sobering truth about Hogan’s health history. “We faced a life-or-death head bleed with Hogan,” says Corby. “The reality is that anyone could die from a head bleed if it’s not treated early enough.”

Head bleeds can occur in children and adults with bleeding disorders. The obvious ones are a result of trauma, such as a fall or injury to the head, but some occur spontaneously from no apparent cause. Classified as one of the five major sites of life-, limb- or function-threatening bleeds by the National Hemophilia Foundation (NHF), intracranial hemorrhages need to be recognized and treated promptly.

Signs and Symptoms in Newborns

The problem with recognizing head bleeds is that symptoms can vary. “Newborns with intracranial hemorrhage may not have any symptoms or minimal symptoms,” says Jeanne Lusher, MD, director of the Hemophilia, Hemostasis and Thrombosis Program at Children’s Hospital of Michigan in Detroit, and the Marion I. Barnhart Hemostasis Research Professor at Wayne State University School of Medicine. “On the other hand, they may be quite dramatic, with neurologic symptoms, lethargy, even shock if they’ve had enough blood loss into the head.”

Routine vaginal deliveries can cause trauma to the head of newborns with hemophilia. “If the baby has severe hemophilia, little bridging veins in the brain can break. Once they start bleeding, they keep on bleeding,” says Lusher.

Lusher advocates distributing specific discharge instructions to all parents of newborns who have a bleeding disorder. “Not to alarm the parents if there are no obvious signs of an intracranial hemorrhage at birth, but for education about the signs,” she says. The instructions should list symptoms parents should watch for in the baby, including excess irritability and sleepiness, irregular breathing, seizures, vomiting and difficulty feeding. Additionally, parents should be told how to measure head circumference—a rapid increase could indicate that the baby’s head is enlarging with blood.

If a baby develops any of these symptoms or isn’t acting normally, Lusher advises parents to stay in close contact with their hemophilia treatment center (HTC). “Call your HTC or go in,” she says. “If the baby has symptoms of an intracranial hemorrhage, we would give a dose of clotting factor and get a CT scan or even an ultrasound of the head.” Although ultrasound is easier to perform—it doesn’t require immobility or sedation—it is not as sensitive as a CT scan.

Lusher was the chair of NHF’s Medical and Scientific Advisory Council (MASAC) when it developed standards of care for newborns with hemophilia who sustained head bleeds. Among the recommendations in the 1998 document, MASAC Recommendation #77 and Medical Advisory #311, “MASAC Recommendations Regarding Neonatal Intracranial Hemorrhage and Postpartum Hemorrhage” are that “all infants with unexplained intracranial hemorrhage should have an appropriate work-up for a bleeding disorder after consultation with a pediatric hematologist” and that vacuum extraction and forceps should not be used in the delivery of babies of known hemophilia carriers.

In a 1999 white paper published in Haemophilia that Lusher co-authored, she and her colleagues discussed the fact that neonatal intracranial hemorrhage can be the initial indicator for hemophilia, which providers often overlook. The authors recommended that written guidelines be established by neonatologists, obstetricians and pediatric hematologists for managing newborns with hemophilia and for neurologic assessment of both term and pre-term babies. The MASAC guidelines go a long way to help, says Lusher, but more must be done. “No national guidelines have been established yet,” she says. “There is a need for approaching the risk of intracranial hemorrhage in neonates in a systematic manner.”

Head Bleeds in Children

“Trauma is a common event in childhood,” says Guy Young, MD, director of the Hemostasis and Thrombosis Center at Childrens Hospital Los Angeles. If you have head trauma and hemophilia, that’s going to put you at risk for intracranial hemorrhage.” He says head trauma in toddlers typically occurs as a result of falls—from stairs, couches, and chairs or highchairs. For older children, head bleeds are typically caused by sports-related accidents and falls—typically off bicycles, skateboards and playground equipment.

For children with bleeding disorders, risk factors for intracranial hemorrhage include diagnosis of severe hemophilia A or B or factor V, X or XIII deficiencies, the presence of an inhibitor and being younger than one year.

Symptoms of a head bleed may not be obvious; there may not be a visible bump or bruise. But there are some symptoms that parents should not ignore. “In a child with hemophilia who suddenly develops a headache that is significant, with vomiting for no apparent reason, seek medical attention immediately,” says Young. The symptoms can escalate in severity, progressing to seizures, lethargy, sleepiness and, eventually, coma. “If you can’t wake up a child, that is very concerning,” he warns.

But a lack of symptoms should not be misinterpreted, says Young. In some rare cases, head bleed symptoms can take several days to develop. “There may be a small amount of bleeding or small blood vessels leaking, so it may take time for enough pressure to build to lead to symptoms,” says Young. “The symptoms are all directly related to the pressure in the brain.”

If any of these symptoms occur, parents should contact their HTC immediately. “Generally what we’ll say is, ‘Go to the emergency room (ER), but give factor first at home,’” says Young.

Once at the ER, the child will undergo tests to determine the location and extent of the bleed. “The mainstay of head trauma is to give factor first and get a CT of the head,” says Young. The physician will also perform physical and neurological examinations of the child. The physical exam can reveal the presence of a bruise or hematoma (a collection of blood from ruptured blood vessels) or swelling. The doctor may test the child’s strength, sensation and vision.

The neurological exam helps determine the child’s level of cognitive ability and consciousness. “In a younger child, I point to one of the parents and ask, ‘Who is this?’ or to a toy that the child can identify,” says Young. “In an older child, I ask him to tell me his name, where he lives and his phone number.” By asking such simple questions, the physician can catalogue the child’s level of awareness and interaction with others; if he is able to communicate; if his memory is intact; and if his attention span has been affected.

Brain Anatomy 101

The brain and its protective membranes are contained in an enclosed space by the skull. So any irritation, such as bleeding or swelling, can cause the brain to expand and pressure to build.

“Subdural hemorrhage is the type usually seen with trauma,” says Young. A child who is riding a bicycle or skateboard without a helmet and hits the pavement at a relatively high speed can sustain this type of head bleed. “If it is a large enough hemorrhage, it could create enough pressure in the brain that other parts could be affected,” says Young. “Subdurals have the potential to lead to brain damage in the local area where the bleeding is occurring.” The damage could include paralysis, vision loss, difficulty speaking, weakness on one side of the body and strokelike symptoms.

For significant subdural hemorrhage, neurosurgery might be required. “Since the bleeding is in an enclosed space, a neurosurgeon may need to make a hole in the skull or remove part of it to allow the pressure to be released,” says Young. That gives the brain needed space to expand and restores blood flow to other areas of the brain. In addition, the neurosurgeon may try to remove the blood that is causing irritation and damage.

“Spontaneous bleeds into the head tend to be intracerebral—within the brain tissue itself,” says Young. They can result from arteriovenous malformations, congenital weaknesses in blood vessels in the brain that cause them to rupture, resulting in bleeding and widespread swelling. Because the bleed is deeper in the brain, it is not as easily accessed. “You may still need the neurosurgical procedure to relieve the pressure, but you’re not going to be able to remove the blood,” Young says. Significant local brain damage can result from this type of head bleed.

Precaution and Prevention

Protecting the head seems logical, especially in toddlers with severe hemophilia who are learning to walk. But not all healthcare providers subscribe to the “helmet every head” ­philosophy.

“If a child is on prophylaxis three times a week and the risk of intracranial hemorrhage is very small, then there’s no need to wear a helmet,” says Young. “It stigmatizes him.”

But Young makes exceptions. “For a toddler with an inhibitor engaging in an activity that may lead to trauma, like going to the playground, I would suggest a helmet.” He also recommends helmets and protective gear for activities that have a high rate of injury in the general population. “Any child who’s riding a bicycle, skateboard or scooter needs to wear a helmet,” says Young. “In many states, it’s a law. Helmet rules apply to all children—with or without hemophilia.”

Recommending helmets for children with hemophilia who want to play certain sports depends on several issues, says Young. “For older children playing sports like baseball and soccer, I don’t recommend wearing helmets unless they’re required for the sport, such as batting in baseball or softball.” But a child with hemophilia who wants to be a soccer goalie needs to take extra precautions. “In this situation, having him wear a helmet is not a bad idea.”

Choosing safe sports is vital for children with hemophilia, especially where the risk of head bleeds is heightened. “NHF’s Sports and Exercise booklet is a useful guide for parents. They should look at it,” say Young. In it, sports and activities are ranked by risk of injury on a color-coded scale from 1 to 3, where 1 is safe and 3 is dangerous and not recommended by NHF.

“There are only a handful of sports I consider taboo: tackle football, wrestling, boxing, ice hockey and lacrosse,” says Young. For high-level competitive sports, such as soccer, he talks with patients about preventive strategies. “I tell them that if they’re going to play competitively, there is a risk of blunt trauma—of butting heads with an opponent. The preventive strategy is that they have to infuse factor before the game,” says Young. That may mean daily infusion throughout the season. That way, the player has adequate factor product in his system—virtually the same as other players on the field—and the risk of a head bleed is decreased.

Teens Will Be Teens

Parents of teens with bleeding disorders would like to believe that Jared will never ride a scooter without a helmet and that Jenny will always wear a seatbelt when she catches a ride with friends. But teenage independence and rebellion manifest in many forms, one of which is risk taking.

“Injuries tend to happen when teens are doing things they know their parents don’t want them to do but they want to be one of the guys or girls,” says Nancy Roy, MSN, FNP-C, nurse coordinator at the Hemophilia Center of South Carolina, an HTC in Columbia. “In younger teens, it can be riding bikes without helmets or skateboarding without protective gear. In older teenagers, it might be a night of drinking with friends. They bang their head, are unaware of it and don’t come into the clinic until a couple of days later. By that time, they have a major head bleed.”

Communication and education are essential components of head bleed prevention, says Roy. “I try to make things as nonthreatening as possible, such as using the American Academy of Pediatrics (AAP) guidelines,” she says. “That way if a child knows that this is something that’s recommended for everybody, regardless of their hemophilia, it does make it a bit more acceptable.” (See sidebar “Using Your Head: Choosing Safe Sports and Activities.”)

The HTC staff encourages open communication with patients before children hit the teenage years. That way, parents can tell their children to call the HTC and see if the sport or activity is approved.

“We try to have time alone with the teen so they can tell us anything they want to,” says Roy. She is blunt, she says, because it is vital that she knows what they are doing. “I tell them, ‘Don’t tell me what you think I want to hear. Tell me the truth and let’s start from there.’”

If a teen has had a head bleed and is on prophylaxis, once he starts feeling better he may stop taking it and start taking risks, says Roy. Some teens have rejected her advice, saying, “I don’t care what you told me.” But many teens do listen to the HTC staff. “My experience has been that they ultimately come to the decision that maybe this isn’t the right thing for them,” she says.

The good news, she says, is that by the time children with hemophilia grow out of their teenage years, things settle down. “By their early 20s, they have figured out a lot of things,” says Roy. “The period of independence is a transitional period and most come through it OK.”

Intracranial Hemorrhage in Adults

In the past, HIV was linked with a higher incidence of spontaneous, nontraumatic intracranial hemorrhage in people with hemophilia. A study in the American Journal of Hematology in 2001 found that older white patients with hemophilia and HIV had a higher incidence of spontaneous intracranial hemorrhage, possibly due to thrombocytopenia, (a low platelet count) that was occurring in white and Hispanic patients and was associated with their HIV infection.

“There was a higher risk of intracranial hemorrhage in adults in the 1990s when HIV was untreated or uncontrolled,” says Sue Kovats-Bell, RN, BSN, nurse coordinator at the Hemophilia Clinic of West Michigan Cancer Center in Kalamazoo. “Now, most of our patients are on HIV medication combination therapy, which is doing a marvelous job of making their HIV disease undetectable.”

Despite better treatments available now, adults are not immune to head bleeds. Spontaneous head bleeds are less common, but they do occur, usually as a result of an aneurysm, cardiac disease, meningitis or stroke from underlying hypertension. They also can occur in patients with inhibitors. Traumatic head injuries that can cause intracranial hemorrhage in adults include motor vehicle accidents, sports injuries and falls.

Symptoms of intracranial hemorrhage in adults are similar to those in children—headache, nausea and vomiting, and sleepiness are all potential indicators. “Some adults may not have experienced a head bleed before,” says Kovats-Bell. The danger is that they may not recognize it or may not infuse enough factor at home. “If you’ve sustained an injury, have an unusual headache or have high blood pressure and other issues that might predispose you to intracranial hemorrhage, don’t hesitate to call your HTC,” she says.

Life After Head Bleeds

Guy Law is 23 years old. As a young child, he experienced two intracranial hemorrhages—one at 13 months old, the other at 30 months. After the first one, he lapsed into a weeklong coma. To relieve pressure in his brain, the neurosurgeon removed most of Law’s left frontal lobe. The procedure caused some long-term complications.

“When I came out of the surgery, I had to relearn how to walk and adapt to my vision changes,” says Law, of Erie, Pennsylvania. “I have no peripheral vision in my left eye and 90% vision loss in my right.” That means Law cannot drive. He wears glasses and has graduated from reading large-print books to reading regular-sized print. “Now I can read anything, sometimes better than my parents,” he says with awe.

Growing up, Law says his parents were overprotective. They encouraged him to wear a helmet until he was 12 and discouraged him from playing most sports. “I didn’t really mind it, because I didn’t want to get hurt,” he says. “I was allowed to go bowling, and I played baseball until I was seven years old.”

Law wears a MedicAlert bracelet to indicate that he has seizures, a result of the brain surgery when he was a toddler. “I’ve had a couple of small ones since I had a grand mal seizure in 1993,” he says. “I just take my meds and pray that I don’t have another seizure. They don’t hinder my life.”

Now living at home with his family, Law works with mentally challenged individuals, is an active member of NHF’s National Youth Leadership Institute and is a senior camp counselor at Camp Hot to Clot. He plans to return to college soon. “I want to go into the medical field and work in the hemophilia community,” he says. “That’s my passion.”

“The brain has a tremendous ability to heal if the intracranial hemorrhage is detected early and treated promptly,” says Kovats-Bell. “Don’t be afraid that it’s a life sentence.”