Jonathan Seaman, 7, was 3 ½ years old when his tonsils and adenoids started acting up. Monthly throat infections, including strep, were treated with rotating rounds of antibiotics. A sleep study confirmed a third problem—sleep apnea. Abnormal results of routine blood tests before adenotonsillectomy, surgical removal of the tonsils and adenoids, led to a fourth diagnosis from a hematologist.
“He tested Jonathan for hemophilia,” says his mother, Dana, 28, a nursing student from Brick, New Jersey. “That’s when we found out he had mild hemophilia A.”
Although adenotonsillectomy is a common pediatric procedure, there can be complications for children with hemophilia or von Willebrand disease (VWD). Special precautions need to be taken prior to, during and after the surgery to curtail bleeding and manage fluids. That’s why it’s important for the pediatric hematologist at your hemophilia treatment center (HTC) to work with your child’s pediatric otolaryngologist (ear, nose and throat, or ENT, specialist) to devise an individualized treatment plan.
Where They Are, What They Do
Tonsils are masses of lymphoid tissue in the mouth where lymphocytes, white blood cells that help the body fight infection, are stored. They contain crypts, indentations that trap harmful microorganisms, such as bacteria and viruses you inhale or ingest. However, tonsils can become infected. Tonsillitis is inflammation of the palatine tonsils on either side of the back of the throat. The term “adenoids” refers to enlarged pharyngeal tonsils high in the throat behind the nose. They are not easily visible without a special instrument.
Troublesome Tonsils
Between the ages of 3 and 4, Jonathan’s tonsillitis worsened. “He had a mix between strep and general throat infections,” says Dana. “He had 10–12 infections that year.” Although the pediatrician prescribed different antibiotics monthly, Jonathan’s parents had concerns. “We were buying antibiotics that were $30 and really strong because the others weren’t working.” They worried about possible side effects and bacterial drug resistance.
When the adenoids are so large that they block the nasal passages and airways, sleep is disrupted. Mouth breathing, snoring and sleep apnea, which is short periods of breathing cessation, can occur. Jonathan’s noisy night breathing would jar his parents. “He was always snoring. My husband would go in a few times a night and flip him on his side if he was on his back,” Dana says.
Obstructive sleep apnea at night causes daytime trouble, too. “Jonathan always used to be very cranky,” Dana says. After school, he would fall asleep if he wasn’t kept busy. Sleep apnea can cause behavioral issues, poor school performance, bed wetting and delayed growth in children.
Enlarged or infected adenoids can also affect the ears. Chronic fluid in the eustachian tubes, which connect the nose and ears, can lead to hearing loss. Caylee Yarnell, 8, was 4 when she started getting strep throat. At the same time, she started talking loudly, but her parents dismissed it. “We have people who are naturally loud in our family,” says her mother, Carrie, 32, a clinical laboratory technologist at The Ohio State University in Columbus. The family lives in South Vienna, Ohio.
The day Caylee sidled up to the TV and placed her ear on the screen, Carrie had an inkling something was wrong. “When the ENT tested her, she was hearing at only 20%. She was hearing yelling only as a whisper.”
Criteria for Surgery
In January 2011, the American Academy of Otolaryngology-Head and Neck Surgery issued the first set of guidelines for clinicians to determine which children are candidates for tonsillectomy. The recommendations include: watchful waiting if a child has recurrent throat infections; taking into consideration allergic reactions to antibiotics, prevalence of fever or history of peritonsillar abscess (pus around the tonsils); and the degree of disruption caused by sleep apnea.
The guidelines offer the number of throat infections that would qualify a child for surgery. “The new guidelines say that the child should have a minimum of three episodes a year for three years or five episodes for two years or seven in one year,” says Margaretha Casselbrant, MD, PhD, chief, Division of Pediatric Otolaryngology, Children’s Hospital of Pittsburgh. However, the episodes are not restricted to strep throat: “They can have a sore throat with fever or with huge tonsils and white exudate (spots).”
There is reason for caution when considering the surgery. “Tonsils and adenoids are a big operation in small children, regardless if they are healthy or have underlying disorders,” Casselbrant says. Further, each child, especially one with a bleeding disorder, needs to be considered individually. The risks and benefits should be discussed with the parents before a decision is made, she says.
Still, the surgery has proven results. “In children with sleep apnea, about 95% are cured by taking out the tonsils and adenoids,” Casselbrant says.
Pre-op Testing
To differentiate obstructive sleep apnea from snoring, parents may have to provide evidence. “When I’m not sure if it’s sleep apnea, I ask the parents to make a tape recording and bring it to me so I can listen,” Casselbrant says. Some physicians may request a videotape of the child sleeping.
“For a child with a bleeding disorder, I would definitely have a sleep study done first before making a decision about surgery,” says Casselbrant. The sleep study, or polysomnography, records the number of times per hour that a child stops breathing for 10 seconds or longer. It also picks up episodes of hypopnea, which is slow, shallow breathing, and measures the oxygenation of the blood. The result is the apnea-hypopnea index (AHI), tabulated by dividing the number of apnea and hypopnea episodes by the number of hours of sleep recorded. According to the American Academy of Family Practitioners, an AHI of greater than 1 and oxygen saturation of less than 92% in a child are considered abnormal.
Jonathan’s sleep study results revealed the extent of his apnea. “He stopped breathing 10 times a night on average,” says Dana. That sounds like an alarming number, but some children with obstructive sleep apnea stop breathing up to 60 times per night, according to the American Academy of Otolaryngology-Head and Neck Surgery.
Other tests your doctor might order include X-rays to determine the size and shape of the tonsils and adenoids, and a throat culture or strep test.
Taking a detailed family and medical history of the child provides critical pieces of information about the possibility of an undiagnosed bleeding disorder. “It’s important to ask the questions: Do you have any bleeding disorders in the family? Does the child bruise easily? Is bleeding prolonged?” Casselbrant says.
Carrie noticed one suspicious symptom, which she shared with the ENT. “I said, ‘It might be the medical person in me, but Caylee does tend to bruise really easily.’” Caylee, not a “girly-girl” but a “rough, tough kid,” according to Carrie, had bruises in odd places, such as on her back or stomach.
Blood tests help confirm the presence of a bleeding disorder. “If there is any concern at all that a child has a bleeding disorder, or there’s a family history of one, then a CBC (complete blood count), a PT/PTT (prothrombin time/partial thromboplastin time, two clotting tests), some screen of platelet function and von Willebrand studies could be done,” says Amy Dunn, MD, pediatric hematologist/oncologist at the Aflac Cancer Center and Blood Disorders Service, Children’s Healthcare of Atlanta. The tests would be modified if there was a history of a bleeding disorder, she says.
“If the PTT is prolonged, that’s when hematologists typically get involved and we proceed further,” says Dunn. However, the PTT is not a reliable diagnostic tool for von Willebrand disease (VWD): “It is not always prolonged in patients with VWD.” Further, stress can release von Willebrand factor (VWF) into the bloodstream. “Because blood draws in young children are often stressful, the VWF levels can double, triple and sometimes quadruple based on stress,” Dunn says.
Caylee’s first blood test came back abnormal. “They did further testing, and that’s how Caylee’s VWD, type 1, was diagnosed,” says Carrie. Family testing then revealed that her husband had low VWF levels and her youngest daughter also had type 1 VWD. This scenario is common, says Casselbrant. “We find quite a high number of patients with VWD picked up here through the testing.”
For a child newly diagnosed with VWD, there are multiple treatment options, including DDAVP or a VWF-factor VIII concentrate. Patients can respond differently to these treatments, so it is important to test an individual’s response before surgery. DDAVP is the treatment of choice in type 1 VWD. DDAVP is a synthetic version of the hormone desmopressin, which releases stored VWF and factor VIII (FVIII), a clotting protein, into the blood. “You give the child DDAVP, then you want to check one and two hours later to see if the levels rise and stay up persistently,” says Dunn. “We had to postpone the surgery so Caylee could go through the DDAVP challenge,” Carrie says. Caylee responded to it, so it was recommended for the surgery. For patients who do not respond to DDAVP, a VWF-containing factor product is used instead, Dunn says.
DDAVP does not work in patients with severe and moderate forms of hemophilia A, but some patients with mild hemophilia A may respond to it. Jonathan’s family took him to the UMDNJ-Robert Wood Johnson University Hospital, their HTC, to gauge his response to DDAVP. Because he did not respond to it, a FVIII concentrate was tried a week later. “Jonathan responded well to that,” says Dana, so it was approved for his surgery.
Treatment Plan
“Our job, in my mind, is to determine what is the appropriate bleeding treatment plan and how we’re going to follow up that patient,” Dunn says. The plan tells the medical staff if factor concentrates or DDAVP are needed and how often to give them. It may recommend the extended use of antifibrinolytics, products that prevent the body from breaking down clots prematurely.
Further, it may describe in detail the necessity for restricting fluids, and monitoring sodium levels and urine output in the child. This is done in patients with VWD who are on DDAVP, which causes the kidneys to retain water, resulting in a dilutional hyponatremia, a low concentration of sodium in the blood. “The risks of hyponatremia with repeated doses of DDAVP are high, especially in young children,” Dunn says. “It is incumbent upon us as hematologists to make sure surgery and anesthesia are not giving a lot of excess fluids, but giving the right type of fluids so kids don’t develop hyponatremia.”
The treatment plan also describes the care the child will receive at home. Some children are sent home with an IV; others get by with the nasal form of DDAVP. Families need to know that some of the heaviest bleeding can occur when the scab sloughs off, up to 10 days after surgery. That may be the time they need to schedule a follow-up office visit. “It’s the second highest period of time where we see bleeding in patients with VWD. We don’t want them to just go home and have no follow-up,” says Dunn.
Surgical Procedure
The best place for a child with a bleeding disorder to have tonsil and adenoid surgery is at a children’s hospital, Casselbrant says. “It should be done by an experienced pediatric otolaryngologist at a hospital with an ICU.” Input from pediatric specialists is needed throughout the procedure, especially from a pediatric hematologist, she adds. The risks of complications for children with bleeding disorders are smaller, she believes, when these specialists are on hand.
The adenoids and tonsils can be removed in a variety of ways. Electrocautery employs a heated metal probe; coblation, radio frequency energy. Cold steel uses stainless steel scissors or a scalpel. Microdebriding involves a rotary cutting tool that shaves the infected tissues. Some studies have shown it can decrease pain and bleeding. Other methods are also used.
“There is no method—whether it’s coblation or cold technique or cauterization—that has truly shown that it is better in children with hemophilia or other bleeding disorders,” says Casselbrant. Depending on their training, surgeons use either coblation or cautery at her institution.
Adenotonsillectomy is performed under general anesthesia and takes about 30–45 minutes. Pediatric patients may be kept overnight, sometimes in ICU for close monitoring. Those with bleeding disorders tend to stay longer, from a few days to a week.
Post-op Care and Complications
The main complication for children with bleeding disorders after adenotonsillectomy is bleeding. Bleeding can occur within the first 24 hours after surgery or days later. Patients receiving DDAVP are also at risk for developing hyponatremia.
“Antifibrinolytics are extraordinarily important in these types of surgeries,” Dunn says. “They remove the tonsil and there’s really nothing to sew together. So you have this open wound and lots of fibrinolysis in the GI tract.” Caylee was on the antifibrinolytic Amicar for 10 days post-op, which is remarkable considering its taste. “Amicar tastes lousy and you have to give it three to four times a day, but most families can get it done,” says Dunn.
Dunn co-authored a literature review in 2010 in Haemophilia showing that the risk of hyponatremia was high in patients with VWD after tonsil and adenoid surgery. Even with fluid and sodium monitoring, more than half of the pediatric patients had mild hyponatremia. “You can’t just use DDAVP without monitoring and without being very cautious of the fluids,” she says.
DDAVP management at home depends on several factors, says Dunn: the child’s response to it, his or her age, whether he or she is on nasal spray or IV treatment, how far the family lives from the HTC and how capable they are. If an IV is in place, a home care nurse may be needed.
Diet restrictions are typical after adenoids and tonsils are removed. “Jonathan couldn’t eat anything crunchy, sharp or hot,” says Dana. Safe foods that are easy to swallow include scrambled eggs, applesauce, pudding and JELL-O®.
Activity is also discouraged while the wound heals. “There was no jumping, running or playing outside for two weeks,” says Dana. To be safe, the Seamans added an extra week of quiet play for Jonathan.
Life After Surgery
Surgical outcomes vary, depending on the child. “Jonathan hasn’t had any throat infections since the surgery,” Dana says. His sleep and mood have improved, too. “He sleeps 12 hours now a night.” But Jonathan had one unforeseen complication: He developed an inhibitor, an antibody to FVIII. This is a rare complication in patients with mild hemophilia A.
Caylee’s snoring and sleep apnea are gone. “She’s an excellent sleeper now,” says Carrie. Her hearing returned to normal after ear tube surgery, which was done at the same time as the adenotonsillectomy. Caylee has had two bouts of strep throat in the past year. The difference is their severity. “When she would get strep before, she would have a super-small opening in her throat,” Carrie says. Now, the sore throat is tolerable. “She is still able to eat and swallow, whereas before there was no eating or swallowing with it.”
Despite their mixed results, both children are breathing, sleeping and swallowing better. “The quality of life of the child will improve when there are proven indications for doing the surgery,” says Casselbrant.
LEARN MORE
- Find out about adenotonsillectomy on the American Academy of Otolaryngology—Head and Neck Surgery Web site: www.entnet.org.
- Dunn AL and Gill JC. Adenotonsillectomy in patients with desmopressin responsive mild bleeding disorders: A review of the literature. Haemophilia 2010; 16: 711-716.