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TJ USED TO really like going toschool. Even though he missed a fewdays due to infections caused byX-linked agammaglobulinemia (XLA),his intraveous immune globulin (IVIG)infusions have kept him healthyand active in the school community.However, at the start of high school,TJ didn’t enjoy school as much,particularly PE. He had not grown asquickly as the other boys in his class,his voice was still high and he had yetto need to use a razor. And, while hewaited for changes to other parts ofhis body, other boys had already seenthose changes in themselves.XLA had delayed the beginning of

puberty for TJ. For boys, puberty beginsat around 9 years of agewhen the brainsignals the release of gonadrotropin-releasing hormone (Gn-RH) into thebody. The Gn-RH causes the pituitarygland to release two other hormones,luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Thesestimulate the sex organs to producesex hormones, estrogen in women andtestosterone in men. These two hor-mones are responsible for sparkingthe development of adult male andfemale characteristics and the changesof puberty.Two puberty disorders are common

to both boys and girls, though.Precocious puberty, or early onsetpuberty, occurs when hormonalimbalances cause puberty to come

early. Delayed puberty,a possible result ofimmunodeficiency, occurswhen puberty comeslater in the teen years.

Precocious PubertyIn precocious puberty,

the process of pubertybegins prematurely, beforethe age of 7 years ingirls and 8 or 9 in boys.Precocious puberty maycause growth of pubicor underarm hair, acneand “mature” body odor.For girls, it may also cause breastdevelopment and the onset ofmenstruation; for boys, it may causegrowth and development of the penisand testicles. Children also may exhibita growth spurt. However, becausetheir bones do not grow fully, they willultimately be passed by their peers,and they will remain short as adults. Psychologically, girls may become

confused or embarrassed about thephysical changes in their body andbecome moody or irritable; boys maybecome more aggressive and developan inappropriate sex drive. Teasingfrom other children may lead tosocial and emotional problems suchas low self-esteem, depression andsubstance abuse.There are two forms of precocious

puberty. In central precocious puberty,

the process simply begins too soon.Some causes may be a process in thebrain present at birth such as hydro-cephalus, excess fluid buildupagainst the brain, or hamartoma, anoncancerous tumor near the brain.Other causes may be a brain or spinalcord injury or exposure to radiation.Certain diseases may cause preco-cious puberty such as McCune-Albright syndrome, congenital adrenalhyperplasia, which is abnormaladrenal hormone production by theadrenal glands, or hypothyroidism, inwhich the thyroid does not produceenough hormones.Less common than central preco-

cious puberty, peripheral precociouspuberty occurs without the involve-ment of the hormone Gn-RH. This iscaused by the release of testosterone

Helping Kids Deal with Delayed Puberty Immune deficiencies can cause delayed puberty in children, but medicine can help and, moreimportantly, parents can help their kids get through that trying time.

By Mark T. Haggard

Parenting:

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or estrogen into the body because ofproblems with sex organs. Peripheralprecocious puberty in both boys andgirls may be caused by a tumor in theadrenal glands that secretes estrogenor testosterone, or, more commonly,exposure to external sources of estro-gen or testosterone such as creams,ointments or chemicals from theenvironment. Ovarian cysts or ovariantumors may start puberty in girls; inboys, early puberty may be the resultof a tumor in the cells that makesperm (germ cells) or testosterone(Leydig cells), or by a rare genemutation called “familial gonadotropin-independent sexual precocity,”which causes early testosterone pro-duction in boys between the agesof 1 and 4. Girls are at higher risk than boys for

precocious puberty. Because fat is animportant catalyst for puberty, thosewho are overweight are at risk. Fatacts as a storage site for sex hormones,so that those obtained from medica-tions or environmental sources maybe kept at higher levels for longerperiods of time, and increase the

likelihood of early pubertal changes.To help reduce the chances of earlyonset puberty, parents should keepchildren away from external sourcesof estrogen or testosterone. They also should help children maintain ahealthy diet and weight. To treat earlypuberty, doctors may prescribe med-

ications such as LHRH analog, asynthetic hormone that suppressesthe pituitary hormones; girls may beprescribed drugs that block estrogenproduction, such as testolactone.

Delayed PubertyAs opposed to precocious puberty,

delayed puberty occurs when theprocess starts later in life. Most ofthe time, delayed puberty is a“constitutional delay,” which meansthe child might simply be a “latebloomer.” This is usually geneticallyinherited from the family.Puberty also may come late as a

result of a medical condition. Immunedeficiencies are specifically linked todelayed onset puberty: e.g., XLA withgrowth hormone deficiency and x-linked severe combined immunodefi-ciency with reduced insulin-likegrowth factor activity (these malechildren are not very responsive togrowth hormone). Children withother immune deficiencies may findpuberty delayed as well, becausesickness makes it harder for theirbodies to grow and develop. Proper

treatment and control over theseconditions can make delayed pubertyless likely. For example, girls who areextremely active may not start pubertybecause they are so lean and theirbodies require a certain amount offat to start puberty.There are two different conditions

that cause delayed puberty. In hyper-gonadotropic hypogonadism, whichcan result from frequent infections,autoimmune problems or as a sideeffect of certain medications, thebrain senses that the ovaries or thetesticles are not making pubertyhormones and sends repeated signalsto the sex organs to try to get themto work. The result is that the LHand FSH are high, but estrogen ortestosterone levels are low. Inhypogonadotropic hypogonadism,which may result from chronic illness,the brain does not make the hor-mones necessary to start puberty. Theresult is that both LH and FSH arelow, while estrogen and testosteronealso are low. The sex organs havethe ability to work normally, butthey do not receive the messagesfrom the brain to begin makingpuberty hormones and, thus, delaypuberty.

Helping Children with Puberty IssuesFor children who are considerably

behind their peers, a pediatricendocrinologist may offer hormonetherapy in an effort to try to “jumpstart” puberty. This course may beenough to get puberty hormonesgoing. “Puberty is an arduous processfor adolescents when normal, but itis more difficult in children withaberrant puberty,” says Dr. Richard D.Blondell of the University of Louisville.“Many physical and biochemicalproblems associated with disorders ofpuberty may be successfully treated.These children benefit from manage-ment by a knowledgeable and sen-sitive clinician.”Beyond medical help, Dr. Blondell

suggests that “psychotherapy can

Immune deficiencies arespecifically linked to delayed

onset puberty.

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play an important role in assistingthese patients as they developphysically and emotionally.” StevenDowshen, pediatric endocrinologistat the du Pont Hospital forChildren in Wilmington, Del., tellschildren: “If you’re feelingdepressed or having school orother problems related to delays inyour growth and development, talkto your mom or dad, your doctoror another trusted adult aboutfinding a counselor or therapistyou can talk to. This person canhelp you sort out your feelings andsuggest ways to cope with them.”Randall Phelps, MD, of theUniversity of Michigan, suggests

that children need to be remindedthat all people go through changes— some go through them earlierand some go through them later.He adds that parents need to givechildren openings to discuss anyworries or concerns, and theyshould encourage kids to take partin regular physical and socialactivities.TJ has now graduated from high

school. He really enjoyed his senioryear, and he is shaving. His parentslistened to him, supported himthrough this difficult time in life andhelped him become a better adult,regardless of his XLA and his late startto adult life. Parents who are there

for their children and are willing tolisten may be enough to settle thefrustration in their children’s hearts. Ifnot, there are doctors willing to help.The connections between delayedpuberty and XLA are clear; the con-nections between early or late puber-ty and other immune deficiencies arenot. Nevertheless, parents activelylistening to their children can seethem through a tough time inbecoming an adult.

MARK T. HAGGARD is a high schoolteacher and football coach, and has threechildren, two of whom have CVID. Heand his wife, Cheryl, also operate Underthe Hood Ministries at www.underthehoodministries.org.

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