Side Effects: Part I

• Common complaints
  
• Neuropsychological testing
  
• Eye and visual problems
  
• Precocious puberty
  
• Diabetes insipidus
  

Common complaints

Most patients with hydrocephalus experience side effects and have some common complaints. How hydrocephalus affects you depends greatly on how your brain and body have reacted to a shunt-related operation.

Hydrocephalus and its treatment can cause a range of common side effects:

• Intellectual (confusion, forgetfulness, or short-term memory problems).
• Visual (eye misalignments or loss of vision).
• Physical (headaches, nausea, or tenderness around incision sites).
• Neurological (seizures or problems coordinating motor skills).
• Endocrinologic (precocious puberty or diabetes insipidus).

Although side effects may be caused by increased intracranial pressure (ICP) or an operation, your neurosurgeon usually won't handle them. Since her job is to operate on the brain and help control hydrocephalus, she may not be trained to handle its side effects. While a majority of neurosurgeons are very proactive about their patients' care, others may be dismissive of complaints of side effects.

My daughter's neurosurgeon is great. She listens to our concerns, calls us out of the blue to check up on her, months after she's had a revision. She's a real sweetheart.

•  •  •   •  •  

I had been having headaches in the evening for about two weeks straight, and it seemed like nothing would make them go away. I finally called my neurosurgeon to ask if it could be a problem with my shunt, but he was quick to write the headaches off as being stress-related. Well, another two weeks went by, and the headaches were still there, and they seemed to be getting worse, too. Rather than call the neurosurgeon and probably be ignored again, I went to the emergency room, where they did a CT, which showed that my ventricles were enlarged.

When side effects arise, you will need to see another specialist such as a neurologist, neuropsychologist, endrocrinologist, neuro-ophthalmologist, or rehabilitation specialist to determine what is wrong and what can be done about it. If your neurosurgeon deals with a lot of hydrocephalus patients, chances are that she will have the names and phone numbers for the best specialists in your area, and can also provide you with a referral.

Neuropsychological testing

Approximately one third of patients who are treated for hydrocephalus will attain a normal intelligence level (i.e., a mean score of between 80 and 100 points on an IQ test) or higher. These patients are more apt to be self-sufficient and capable of leading a normal life. They may, however, have other mental difficulties, including:

• Poor spatial relations.
• Short-term memory problems.
• Difficulty in concentration.
• Non-verbal learning disorder (NVLD).
• Problems with social skills.
• Difficulty processing complex language.
• Learning disabilities.
• Physical limitations and problems with coordination.

Long-term side effects can be caused by the condition itself or as a result of one or many operations on the brain. The sad thing is that many patients and their families aren't told about these side effects.

If you have a neuropsychological examination (neuro-psych exam, for short), the neuropsychologist can identify specific neurological deficits and learning or social disorders. Then, based on the results of the testing and examination, she can identify ways for you to relearn how to perform specific tasks to help make your daily life easier.

Although performed on both children and adults, neuro-psych exams are more commonly given to children. A neuro-psych exam can be given as a baseline, and then if there are difficulties after subsequent shunt revisions, the child can be retested and the scores compared with those of the baseline exam. Armed with the information from these tests, parents and educators can know when to make or change an individualized education plan.

Paying for the exam

Just getting in to see a neuropsychologist can be a battle in itself. A thorough neuropsychological examination can cost up to $2,000, particularly if the findings are reviewed with you afterwards. What an insurance company sees when a referral request for a neuro-psych exam is received is usually the word "psychologist." This makes claims reviewers think that the exam is to study the patient's psychological or mental health, which is untrue. Because most insurance companies don't recognize neuropsychological examinations as a form of treatment for hydrocephalus patients, the approval process may be very difficult.

Our daughter's neurosurgeon recommended that she have a neuropsychological exam following her last shunt revision. At first, the insurance company flat-out refused to cover the cost of the testing. It's been four months now, and we're still going through the appeals process with them, but we're standing our ground because the outcome of the test could have a dramatic impact on Emily's future. If push comes to shove, we'll pay for it ourselves, but the way we look at it, if the neurosurgeon recommends a test, or series of tests, to help Emily recover from her surgery, then the insurance company should agree with the recommendation instead of playing games with our daughter's health.

Before making an appointment with a neuropsychologist, contact your insurance company and ask if your policy will cover the cost of the testing. If your medical insurance company refuses to pay for the neuro-psych exam, remind them that this is a medical evaluation specifically related to surgical problems, and not an exam of the patient's psychological or mental health. If they continue to deny you access to the examination, you could:

• Have your primary care physician (PCP), neurologist, and/or neurosurgeon contact your insurance company stating their reasons for recommending the exam.
• Write a letter of appeal. In your letter, ask them to reexamine their denial for treatment, and give valid reasons why this examination is important to long-term care. (See the section at the end of Chapter 10, Insurance, for sample appeal letters.)

Remember, neuropsychological testing should be included as a part of the care and treatment for hydrocephalus. Be firm with your insurance company about them covering the cost of the examination, and encourage your doctors to take part in the appeal process if one becomes necessary.

How do you benefit from the exam?

The results of a neuro-psych exam pinpoint the specific areas in which you have problems. Results can then be used:

• To help you develop strategies to maximize performance of various tasks.
• To help make you more conscious about the condition and its effects.
• For parents, to provide you with ammunition to deal with schools when developing an individualized education program for your child and to help in making realistic decisions regarding your child's educational needs.
• For adults, to help in making realistic career decisions.

Selecting a neuropsychologist

Time spent selecting a neuropsychologist is an investment in the future. Your neuropsychologist will help identify any deficits resulting from the condition or surgeries and will make specific, practical recommendations on how to address them. The neuro-psych exam will help you chart a course through life based on a clear understanding of strengths and weaknesses. For example, the report will be the basis on which you plan your child's education.

Before starting the neuro-psych exam, meet with or phone prospective neuropsychologists to ask the following questions:

• Are you licensed by the state? If so, what is your license number?
• Are you registered with the American Board of Professional Neuropsychology (ABPN) or the American Board of Clinical Neuropsychology?
• How long have you been in practice?
• Do you prefer to work with children or adults?
• How many hydrocephalus patients have you evaluated?
• How do you determine which tests to give the patient?
• After the testing has been completed, how will your findings be presented to us? Will we receive a written report? Will there be a follow-up session where we can go over your findings and recommendations?

Look for a neuropsychologist who has excellent credentials, an excellent reputation in your community, and experience in evaluating and examining the needs of people with hydrocephalus. At the end of the examinations, and after the neuropsychologist has had a chance to evaluate the data, you should receive:

• A written report or have the opportunity to talk with the neuropsychologist regarding the test results.
• A summary or a full report that details the findings of the neuropsychologist's recommendations.
• A review of that report at length.

What is a neuro-psych examination?

A neuro-psych exam involves a series of tests administered by a licensed neuropsychologist. Neuropsychology is a branch of psychology that focuses on the relationship between brain functioning and behavior. Testing is done to assess brain function, to predict outcomes, and to provide strategies to modify the outcome as needed.

The neuropsychological examination includes different phases. The exam usually begins with a thorough screening of medical records. The neuropsychologist will become familiar with the medical history, including:

• The types of operations performed.
• Complications that may have occurred during surgery.
• Any short- and long-term side effects specifically related to various operations and hydrocephalus.
• Physical problems which are attributable to hydrocephalus, including a lack of coordination, gait disorder, visual impairments, difficulty interpreting speech, and problems with hearing.
• Any medications used for other conditions such as seizures.
• Any other medical conditions.

Once the neuropsychologist has had an opportunity to review all the medical records, the next step is to meet with the family. During this consultation, the neuropsychologist will ask about some of the difficulties experienced on a day-to-day basis.

Results of the neuropsychological examination

Based on the results compiled from the tests, the neuropsychologist can make recommendations for rehabilitation. Some typical recommendations may include:

• Speech therapy to improve written, oral, and audible language comprehension.
• Physical therapy to help rehabilitate motor problems and improve coordination. Physical therapists can help patients by retraining them to coordinate the use of muscles and limbs which may have been weakened or have reduced functionality as a result of damage to the brain.
• Occupational therapy to help train or retrain patients in how to perform certain tasks to maximize independence in daily life.
• Social skills training to help patients learn how interact with other children and adults.
• Social support to help families cope with any short- and long-term care needs.

Believe it or not, it can be better to suffer brain damage as a child than later in life as an adult. This is because the neurons in the brain of a child can assume functions from damaged areas more easily than in an adult because the child's brain is still growing.

Eye and visual problems

Hydrocephalus can weaken the nerves that control eye muscles and visual responses, resulting in eye misalignments and loss of vision. Adults may experience double vision. Children may see double at first, but later develop a "lazy" eye if a prompt diagnosis and treatment isn't made. Additionally, eye and visual problems can occur slowly as ICP increases, and can also occur after a sudden decrease in ICP following a shunt placement or revision.

How the brain sees

Although the eyes are located in the front of the head, the part of the brain that interprets the images--the occipital lobe--is located at the back of the head. The images come in through the eyes, and the information travels along the optic nerves, through the brain, and into the occipital lobe. The occipital lobe translates information sent from the eyes so you know what you are looking at.

Four cranial nerves control eyesight and movements:

• The optic nerve. Also known as the second cranial nerve, the optic nerve is responsible for transmitting visual images from the eyes to the brain. The optic nerve is made up of approximately one million tiny fibers which receive information from the rod and cone cells of the retina.
• The oculomotor nerve. Also known as the third cranial nerve, the oculomotor nerve is responsible for controlling four of the six main muscles of the eye, as well as those of the upper eyelid. The fibers of the oculomotor nerve help control eye movements and reactions of the pupils to light. The oculomotor nerve is rarely affected by hydrocephalus alone, unless there are other problems within the central nervous system.
• The trochlear nerve. Also known as the fourth cranial nerve, the trochlear nerve is responsible for rotating the eyeball while at the same time turning it downward and from side to side.
• The abducens nerve. Also known as the sixth cranial nerve, the abducens nerve is responsible for controlling the lateral rectus muscle of each eye, which turns the eye outward, away from the nose.

These four nerves affect vision in different ways: controlling eye movements, helping to adjust pupil reactions, moving the eyelids, and transmitting the images to the occipital lobe. The way these four nerves are affected by hydrocephalus, however, is the same.

Each of these nerves must travel from the back of the eyeball, through the meninges, and into the brain. When ICP increases, the pressure can be transmitted to the eye nerves, causing visual and eye problems. The problem can be traced back to the individual or group of nerves that supply the eyes.

Symptoms

If left untreated for an extended period of time, visual impairment (including blindness) can be permanent, and eye misalignments become more complex to treat. Therefore, it is important to watch for symptoms associated with increased ICP which affect the eyes. These include:

• Grayed-out or fuzzy vision.
• Double vision (diplopia).
• A reduced field or loss of peripheral vision.
• Misaligned, lazy, or wandering eyes.
• Unstable, shaking eyes.
• Eyes in a downward gaze (mainly found in infants).

If you detect any of these signs and symptoms, contact an ophthalmologist or pediatric ophthalmologist as soon as possible.

Ophthalmologists

An ophthalmologist is a physician who specializes in the diagnosis and treatment of eye diseases and abnormalities. A neuro-ophthalmologist is an ophthalmologist who has additional training in neurology and specializes in treating visual problems that are caused by neurological conditions. Finding a neuro-ophthalmologist may necessitate a call to one of the larger teaching institutions, as the subspecialty is not found at every hospital center.

All patients who have hydrocephalus should have an annual examination by an ophthalmologist to monitor any changes in vision and to diagnose and treat them promptly.

Children who have hydrocephalus should be examined by a pediatric ophthalmologist. Pediatric ophthalmologists, like pediatricians, are specially trained to work with children and infants.

We were having an unusual eye involvement issue after a series of revisions. Shaun's eyelid would droop down whenever he had a headache. Our neurologist suggested we see a neuro-ophthalmologist. He was able to pinpoint that his droop, or ptosis, of the lid was due to a third nerve palsy that would just become more defined when he was not feeling well.

Pediatric ophthalmologists use specialized methods to diagnose problems. For example, younger children are best tested using picture vision tests or by observing tracking behavior for small toys.

The ophthalmologist will look for the following when examining the eyes:

• Swelling of the optic nerve (papilledema).
• Eye misalignments (strabismus).
• Crossed eyes (esotropia) is the most common form of eye misalignments.
• Lazy eye (amblyopia). Amblyopia causes a reduction in vision but is reversible by patching the good eye to improve the lazy eye's condition.
• A reduction of color vision.
• Reduced or loss of peripheral vision (vision to one or both sides).
• Abnormal pupil reaction to light.
• Vision that is less than 20/20.

A reduction in vision is not always the earliest sign of hydrocephalus affecting the eye. Other parts of the eye exam are more sensitive in identifying early eye problems caused by hydrocephalus. Of particular importance to the ophthalmologist is the appearance of the optic nerve. The appearance of the optic nerve is evaluated with a three-dimensional view to look for any swelling or paleness, which would indicate that the optic nerve is damaged as a result of increased ICP.

If papilledema is present, it is a sure sign that the ICP is elevated. However, the absence of papilledema does not mean that the ICP is normal. Most patients with elevated ICP do not have papilledema.

Eye misalignments

Some children with hydrocephalus develop eye misalignments, or strabismus. Strabismus can force your child to assume a head posture with a tilt or turn in one direction to bring her other eye into focus with the misaligned eye. If she is learning to walk, she may be hindered by turning her chin downward or turning her head to the side to reduce double vision. To treat strabismus, your child's ophthalmologist will prescribe patching in an alternating fashion to eliminate double vision. If her eyes fail to realign following a shunt placement or revision, her ophthalmologist may need to perform surgery on the eye muscles to improve the alignment.

A reversible cause of poor vision called amblyopia can occur in children who are less than eight years old when one eye is favored due to strabismus or another cause. The ophthalmologist will try patching the good eye in an effort to get the brain to use the amblyopic eye.

A problem seen in some infants with hydrocephalus is that the eyes are turned downward, restricting the child from gazing upward ("sunsetting" of the eyes). Sunsetting of the eyes normally occurs before children are properly diagnosed and treated for hydrocephalus. A sudden reoccurrence of sunsetting may indicate that the shunt is not working properly.

Impaired vision

Trouble with seeing small items or objects in the peripheral field of view indicates that ICP is probably rising. Visit your ophthalmologist immediately so she can check out your current vision and compare it to the results from previous examinations. In these situations, prior baseline results enable the doctor to note subtle changes in vision or the early stages of increasing ICP.

Perceptual defects

It is fairly common for children with hydrocephalus to have difficulty naming objects, reading, or calculating math problems. An early step in evaluating perceptual defects in your child should include visual and hearing examinations. Your doctor can advise you about additional testing, including formal educational and developmental assessments offered through school or by a medical facility. Many hospitals provide developmental assessments or neuropsychological testing through their department of neurology or psychology.

Perceptual defects can add to a child's frustration at school or an adult's frustration in the workplace. Social situations can become awkward as well. However, these perceptual difficulties can be assessed, and strategies can be learned to compensate for them.

Precocious puberty

Precocious, or early, puberty is a disorder that causes some children to go into puberty prematurely. Although precocious puberty is rare in the general population, it can be fairly common in children between the ages of 5 and 13 years old who have hydrocephalus, myelomeningoceles, cerebral palsy, and microcephaly. It affects both males and females, but occurs more often in girls than boys.

Linda started developing at about five years old. She is nine years old now, and she is showing signs of hormones--PMS. When it first started to happen I thought she was going through shunt malfunction. I believe that it also affects her shunt. She also has hair ( pubic and under her arms) coming in, and her breasts are developing now. She has not, however, gotten her period.

What causes precocious puberty?

Precocious puberty is presumed to be caused by ICP. This increase in pressure can cause damage to the hypothalamus and the pituitary gland, which are responsible for timing the release of gonadotropins and sex hormones into the bloodstream.

Precocious puberty has been linked to the number of shunt revisions a child has prior to reaching the normal age of puberty (10 to 15 years old for girls and 12 to 18 years old for boys). Young children are at greater risk of experiencing precocious puberty if they have had numerous shunt revisions related to increased ICP.

Symptoms

The symptoms of precocious puberty are easy for parents to recognize, as children will begin to develop sexually at an age much earlier than their peers. Girls experience early breast development, menstruation, growth of pubic and body hair, an increase in weight caused by muscle development, and an increase in hip size. Boys experience growth of facial, body, and pubic hair, development of the penis and testes, deepening of the voice, and an increase in weight caused by muscle development.

If you begin to notice these changes in your child, you should make an appointment with your primary care physician (PCP). After examination by your PCP, your child should be referred to see a pediatric endocrinologist, who will order blood tests to check for increased hormone levels in the bloodstream.

Precocious puberty is also responsible for shutting down the growth plates of the bones, causing short stature. The pediatric endocrinologist may order an X-ray of your child's hand to see if your child's bones are aging too quickly.

If caught early enough, precocious puberty can be suppressed by treating the condition with hormone suppressants.

Diabetes insipidus

Diabetes insipidus is an extremely rare form of diabetes that is unrelated to diabetes mellitus (commonly known as "sugar diabetes"). Diabetes insipidus is caused when the secretion of the hormone vasopressin is inadequate, or when the kidneys don't respond to stimulation by the hormone. Vasopressin (also known as the antidiuretic hormone, or ADH) is the hormone that regulates fluid reabsorption by the kidneys.

Many of the same conditions that cause or accompany hydrocephalus can also cause damage to the hypothalamus or the pituitary gland, which produces the hormone vasopressin. Those causes include:

• Meningitis.
• Head trauma.
• Brain tumors.
• Increased ICP.

Symptoms and tests

People who have diabetes insipidus often complain of constant thirst (polydypsia), and frequent urination (polyuria), particularly at night.

I have always gotten up in the middle of the night to go pee, but since my last shunt revision, it was getting much worse--as frequent as six or seven times a night. My sleep was getting disrupted and I always seemed to be tired throughout the day.

Another common symptom for diabetes insipidus is abdominal bloating. People with diabetes insipidus often complain of having a swollen abdomen at night before bedtime, which disappears by morning.

It was awful. I do about 100 sit-ups a day, and I always seem to be bloated. There were some nights where I would look like I was three or four months pregnant before going to bed. Then by morning, I'd have a flat tummy again! I would notice that by midday my tummy would start bloating again. It was embarrassing.

Additional symptoms include dry hands and constipation.

Your doctor or endocrinologist has several methods to diagnose diabetes insipidus. A CT or MRI scan of the head is done to evaluate the pituitary gland. A simple blood test can identify the level of vasopressin in your bloodstream. Another test that can be performed is a water-deprivation test. You will be asked to drink a normal amount of water throughout the day, then medical staff will weigh you frequently over several hours to check on water retention. Any urine you produce will be tested. During the test, your blood will be drawn every hour for several hours to check the vasopressin levels in your bloodstream.

Treatment

If you are diagnosed with diabetes insipidus, your endocrinologist will prescribe desmopressin, a drug more commonly referred to by its trade name, DDAVP. DDAVP is usually prescribed as a nasal spray, but is also available as nose drops and in tablet form. The nasal spray is sprayed into each nostril prior to going to bed and, if needed, in the morning as well. As with all medications, check with your doctor or pharmacist about the proper use and dosage, and be sure to ask if there are any possible drug interactions.

DDAVP

Also called: Desmopressin acetate.

How given: Nasal spray, nose drops, or tablets.

How it works: Prevents or controls frequent urination or loss of water associated with diabetes insipidus by supplementing ADH.

Common side effects: Headache, nausea, mild abdominal cramps, stuffiness or irritation to the nose, flushing.

Infrequent side effects: Nosebleeds, sore throat or cough, upper respiratory infection, cold symptoms (runny nose, chills, etc.), conjunctivitis (pinkeye), depression, dizziness, an inability to produce tears, development of a rash on the legs, swelling around the eyes, and weakness or fatigue.

Possible drug interactions:

• Any drug used to increase blood pressure.
• Clofibrate (Atromid-S).
• Glyburide (Micronase).
• Epinephrine (EpiPen).