Rabson-Mendenhall syndrome is a rare genetic condition that causes extreme insulin resistance. Features often begin in infancy or early childhood and continue lifelong. Many people with Rabson-Mendenhall syndrome have slow growth, high blood sugar, skin changes, and dental differences. Serious complications can occur, and life expectancy may be shortened, but outcomes vary by severity and care. Treatment focuses on careful blood sugar management, nutrition, and specialist support, and researchers continue to study new options.
Overview
Rabson-mendenhall syndromeSymptoms
Rabson‑Mendenhall syndrome causes severe insulin resistance starting in early infancy. Features include poor growth, very high blood sugar with occasional lows, dark velvety skin, dental and facial differences, excess body hair, enlarged genitalia, and risk of dehydration or ketoacidosis.
Outlook and prognosis
Living with Rabson-Mendenhall syndrome often means complex, lifelong care, but supportive treatments can ease symptoms and improve comfort. Growth challenges, blood sugar swings, dental and skin changes, and infections may progress over time. Close, coordinated care helps families plan and adapt.
Causes and risk factors
Rabson-Mendenhall syndrome results from inherited changes in the insulin receptor gene (INSR), usually autosomal recessive. Risk rises when both parents carry a variant, especially when parents are related by blood. Illness, nutrition, and timely care can modify severity and complications.
Genetic influences
Genetics are central in Rabson-Mendenhall syndrome; it results from inherited changes in the insulin receptor gene. Most cases are autosomal recessive, meaning both parents carry a variant. Different variants can influence severity, age at diagnosis, and response to treatments.
Diagnosis
Doctors suspect Rabson-Mendenhall syndrome from early severe insulin resistance, growth delay, and skin or dental changes. Genetic diagnosis of Rabson-Mendenhall syndrome is confirmed by INSR gene testing, alongside labs showing very high insulin levels and glucose abnormalities.
Treatment and drugs
Treatment for Rabson-Mendenhall syndrome focuses on easing insulin resistance and supporting growth, energy, and organ health. Care often includes carefully adjusted insulin, medications that improve insulin sensitivity, nutrition planning, and monitoring for dental, skin, and hormonal issues. A coordinated team reviews needs regularly and adapts treatment as children grow.
Symptoms
From infancy, families may notice slow growth, feeding challenges, and dark, velvety skin patches in areas like the neck or armpits. Rabson-Mendenhall syndrome is linked with severe insulin resistance, so early features of Rabson-Mendenhall syndrome often touch growth, blood sugar, skin, and teeth. Features vary from person to person and can change over time.
Babies may be small at birth and then grow more slowly than peers. Clothes and shoes may fit longer than expected as height and weight lag. Pediatricians may track growth charts closely.
In Rabson-Mendenhall syndrome, the body doesn’t respond well to insulin, so blood sugar can swing between highs and lows. Highs may bring thirst and more trips to the toilet; lows can cause shakiness, sweating, or irritability. These episodes can happen even with regular meals.
Dark, velvety patches can develop on the neck, armpits, or groin. Skin may also feel thicker on the palms and soles, and small skin tags can appear. In Rabson-Mendenhall syndrome, these changes reflect the body making a lot of insulin but not using it well.
Features can look coarser over time, with a broader nose or fuller lips. Some may notice a wider mouth or a more prominent jaw. Photos over months can make changes easier to spot.
How people usually first notice Rabson-mendenhall syndrome?
Parents and doctors often first notice Rabson–Mendenhall syndrome in early infancy when a baby isn’t gaining weight well despite feeding, has very high blood sugars, and shows unusual skin changes like thickened, velvety patches (acanthosis nigricans) around the neck, armpits, or groin. Over time, families may spot additional early clues such as dental crowding, an enlarged tongue, coarse facial features, and abnormal hair growth, which prompt testing for severe insulin resistance. In many cases, the “first signs of Rabson–Mendenhall syndrome” are the combination of extreme insulin resistance in a very young child plus these striking skin and facial features that lead clinicians to consider the diagnosis.
Types of Rabson-mendenhall syndrome
Rabson-Mendenhall syndrome is a rare genetic condition caused by changes in the insulin receptor gene, which disrupts how the body responds to insulin. There aren’t widely accepted distinct clinical variants; instead, severity can vary from person to person and across ages. People may notice different sets of symptoms depending on their situation. Because no recognized subtypes are defined, there are no formal types of Rabson-Mendenhall syndrome to list.
No recognized variants
Experts do not define distinct types of Rabson-Mendenhall syndrome. Differences you read about usually reflect a spectrum of severity rather than true variants. This means discussions of “types of Rabson-Mendenhall syndrome” refer to degree of insulin resistance and complications, not separate genetic subtypes.
What genetics can tell you about symptoms
In Rabson–Mendenhall syndrome, changes in the insulin receptor gene make the body “hard of hearing” to insulin, leading to severe early insulin resistance, high blood sugar, and slow growth. These variants also link to acanthosis nigricans, dental crowding, enlarged organs, and genital differences.
Causes and risk factors
Rabson-Mendenhall syndrome is caused by changes in the INSR gene that weaken the insulin receptor. It is inherited in an autosomal recessive way, meaning a child is affected when both parents pass on a nonworking copy. The main risk factors for Rabson-Mendenhall syndrome are having parents who are carriers and parents who are related by blood. Some risks are written in our DNA, passed down through families. Lifestyle or environmental factors do not cause the condition, but healthy habits can support care and do not change the genetic risk.
Environmental and biological risk factors
Rabson-Mendenhall syndrome is very rare, and current research has not identified environmental exposures or parent-related biological features that reliably raise the chance of it occurring. Doctors often group risks into internal (biological) and external (environmental). Because the condition is uncommon, available studies are small, so what we know is limited. This page focuses on risks rather than early symptoms of Rabson-Mendenhall syndrome.
Advanced maternal or paternal age has not been shown to increase the likelihood of Rabson-Mendenhall syndrome. Current studies do not support an age-related effect.
Common pregnancy conditions such as diabetes, thyroid problems, or high blood pressure have not been linked to higher risk of Rabson-Mendenhall syndrome. No consistent parent-health pattern has been observed.
Infections during pregnancy, including common viral or bacterial illnesses, have not been shown to trigger this syndrome. No specific infection has been tied to a higher chance of Rabson-Mendenhall syndrome.
Exposure to ionizing radiation, heavy metals, or hormone-disrupting chemicals during pregnancy has not been proven to raise the risk of Rabson-Mendenhall syndrome. Evidence to date does not show a repeatable environmental exposure pattern.
Genetic risk factors
In families where severe insulin resistance shows up in infancy, the cause is often genetic. Rabson-Mendenhall syndrome most often stems from harmful changes in the INSR (insulin receptor) gene, which disrupts how cells respond to insulin. Some risk factors are inherited through our genes. Knowing the inheritance pattern can help families understand chances for future children.
INSR gene variants: Changes in the INSR (insulin receptor) gene are the main genetic cause of Rabson-Mendenhall syndrome. These changes blunt the insulin signal from birth, which helps explain early symptoms of Rabson-Mendenhall syndrome. Different variants can affect the receptor in different ways.
Recessive inheritance: A child is affected when they inherit two non-working copies of the gene, one from each parent. When both parents are carriers, each pregnancy has a 25% (1 in 4) chance of Rabson-Mendenhall syndrome.
Carrier parents: Parents are usually healthy carriers with one non-working and one working copy. Each child then has a 50% (1 in 2) chance to be a carrier and a 25% (1 in 4) chance to be affected.
Related parents: When parents are blood relatives, they are more likely to share the same rare INSR variant. This raises the chance of having a child with Rabson-Mendenhall syndrome.
Affected sibling: Having one affected child means the parents are very likely both carriers. Future pregnancies then have a 25% (1 in 4) chance to be affected and a 50% (1 in 2) chance to be carriers.
Variant severity: Different INSR changes can lead to a spectrum of severity. Some variants cause milder insulin resistance, while others cause more severe features within this syndrome.
Lifestyle risk factors
Rabson-mendenhall syndrome is a genetic condition; lifestyle habits do not cause it, but they can influence day-to-day glucose swings, risk of ketosis/dehydration, and comfort with skin and dental symptoms. Thoughtful nutrition, activity, sleep, and self-care can make the metabolic instability easier to manage. Below is how lifestyle affects Rabson-mendenhall syndrome in practical ways. Choose and adapt these with your clinical team.
Large amounts of rapidly absorbed carbs can trigger extreme post-meal hyperglycemia and ketone build-up. Favor lower-glycemic, fiber-rich carbs and added protein/fat to blunt glucose spikes.
Sweetened beverages are absorbed very fast and can sharply raise glucose and worsen dehydration from osmotic diuresis. Replacing them with water or unsweetened options may reduce swings and thirst.
Skipping meals or eating very large, infrequent meals can cause wide glucose fluctuations and ketosis risk. Small, regular meals or snacks can steady glucose and energy levels.
Prolonged inactivity can worsen hyperglycemia by reducing glucose uptake in muscle. Gentle, regular movement—especially after meals—may modestly improve glucose disposal; monitor for fatigue and hydration.
Risk prevention
Rabson-Mendenhall syndrome cannot be prevented because it is genetic, but many complications can be lowered with steady care and planning. Prevention can mean both medical steps, like vaccines, and lifestyle steps, like exercise. Focus on catching early symptoms of Rabson-Mendenhall syndrome, managing blood sugar swings, and staying ahead of dehydration and infections. Regular specialist follow-up helps tailor steps as needs change over time.
See an endocrinology team regularly to adjust care as your child grows. Close follow-up helps prevent severe highs and lows linked to Rabson-Mendenhall syndrome.
Use frequent blood sugar checks or a continuous monitor to spot swings early. Tracking patterns helps reduce emergencies like diabetic ketoacidosis.
Work with a dietitian on steady, balanced meals and snacks to blunt sharp glucose spikes. Tailored plans for Rabson-Mendenhall syndrome can also address growth and energy needs.
Have a clear plan for illness, including how often to check sugars, fluids to use, and when to seek urgent care. Illness can quickly trigger dehydration or ketosis in Rabson-Mendenhall syndrome.
Transmission
Rabson-Mendenhall syndrome is not contagious and cannot be caught or spread through everyday contact. The condition is inherited in an autosomal recessive pattern: a child needs to receive two changed copies of the same gene—one from each parent—who are usually healthy carriers. When both parents are carriers, each pregnancy has a 25% (1 in 4) chance of a child with Rabson-Mendenhall syndrome, a 50% (1 in 2) chance of a child who is a carrier, and a 25% chance of a child with neither; the chance can be higher in families where the parents are related by blood. Very rarely, a new gene change can occur, but the genetic transmission of Rabson-Mendenhall syndrome almost always follows this carrier-to-child pattern.
When to test your genes
Rabson-Mendenhall syndrome is a rare, genetic insulin receptor disorder. Consider genetic testing when a child has severe insulin resistance from infancy or early childhood, especially with features like early-onset diabetes, abnormal teeth or nails, growth restriction, or distinctive facial features, or when there’s a family history. Testing guides care, informs recurrence risk, and enables carrier testing.
Diagnosis
Daily life clues often come first: a child who eats well but isn’t growing as expected, skin that looks darker and velvety in body folds, or blood sugars that swing high and low. Rabson-Mendenhall syndrome is rare, and the diagnosis is based on a pattern of features confirmed by specific tests. Early and accurate diagnosis can help you plan ahead with confidence. When doctors talk about the genetic diagnosis of Rabson-Mendenhall syndrome, they’re usually combining what they see in clinic with lab and genetic findings.
Doctors look for a consistent pattern such as coarse facial features, dark velvety skin in the neck or armpits, and early or unusual dental changes. Enlarged genitals in children and excess body hair may also be present. This cluster of findings points toward Rabson-Mendenhall syndrome.
Height and weight are mapped over time to spot slow growth despite adequate eating. Head size and body proportions are checked for patterns seen in severe insulin resistance. These measurements help time when symptoms began and how they’re progressing in Rabson-Mendenhall syndrome.
Fasting and post-meal glucose are paired with insulin levels to look for very high insulin alongside fluctuating sugars. This pattern supports severe insulin resistance seen in Rabson-Mendenhall syndrome. ... and other lab tests may help rule out common conditions.
A blood test looks for changes in the insulin receptor gene (INSR) to confirm the diagnosis. Finding a disease-causing variant establishes Rabson-Mendenhall syndrome and can guide care and family testing. Genetic testing may be offered to clarify risk or guide treatment.
Stages of Rabson-mendenhall syndrome
Rabson-mendenhall syndrome does not have defined progression stages. Signs start early and vary widely from child to child; early symptoms of Rabson-Mendenhall syndrome can appear in infancy, and complications may surface at different times rather than in set phases. Doctors consider growth patterns, difficult-to-control blood sugars, and certain skin, hair, or dental features; Genetic testing may be offered to clarify certain risks. Ongoing care usually involves regular checks of glucose and ketones, growth and nutrition, and screening of the kidneys, eyes, heart, and teeth to track changes over time.
Outlook and prognosis
Looking at the long-term picture can be helpful. Rabson–Mendenhall syndrome is rare and often severe, so everyday life usually involves frequent medical visits, careful meal planning, and close monitoring of blood sugars. Many people ask, “What does this mean for my future?”, and the honest answer is that the outlook depends on how early the condition is recognized and how well blood glucose can be balanced over time. Even though it can feel overwhelming, small wins—like fewer glucose spikes, better hydration, and good sleep—can add up and support overall health.
Prognosis refers to how a condition tends to change or stabilize over time. In Rabson–Mendenhall syndrome, extreme insulin resistance starts early in childhood and can lead to high blood sugar swings, dehydration, infections, and strain on the heart and kidneys. Serious complications are common, and without advanced care, life expectancy can be shortened, sometimes into adolescence or early adulthood. That said, newer options—such as continuous glucose monitoring, specialized insulin strategies, and targeted therapies offered in expert centers—may improve comfort and day-to-day stability for some children. Early symptoms of Rabson–Mendenhall syndrome often include poor weight gain, dental issues, and very high glucose levels; recognizing these early can open doors to supportive treatments sooner.
Talk with your doctor about what your personal outlook might look like. Because care plans are highly individualized, asking about clinical trials, nutrition support, and infection prevention can help you plan ahead. Loved ones often play a key role in helping with daily care, appointments, and spotting early warning signs of dehydration or illness. Genetic testing can sometimes provide more insight into prognosis, and it can guide family planning discussions as well.
Long-term effects
Rabson-Mendenhall syndrome is a rare genetic condition that changes how the body responds to insulin over a lifetime. Long-term effects vary widely, and no two people follow exactly the same course. What early symptoms of Rabson-Mendenhall syndrome look like in childhood can shift as growth, hormones, and blood sugar demands change. Many live with ongoing issues tied to blood sugar swings and organ stress, while some features remain fairly stable.
The body stays extremely resistant to insulin, so blood sugar can swing from low to very high. Over time this can lead to diabetes-like complications and a higher risk of dangerous acid buildup (ketoacidosis).
Many have slow growth with shorter height and lower weight than peers. Body fat and muscle can be distributed differently, which may become more noticeable with age.
Thick, velvety dark patches (often on the neck, armpits, or groin) can persist and sometimes spread. Skin tags and thickened skin on the palms or soles may also appear over time.
Enamel defects, widely spaced teeth, and jaw differences often persist into adulthood. These features can affect chewing and may influence speech clarity.
How is it to live with Rabson-mendenhall syndrome?
Living with Rabson-Mendenhall syndrome often means planning your day around frequent meals, careful monitoring of blood glucose, and regular medical visits to manage severe insulin resistance. Many children and adults contend with fatigue, gastrointestinal discomfort, and skin changes, while families coordinate specialized care, adapt school or work schedules, and stay alert for emergencies like dehydration or infections. Daily life can feel medically busy, but clear routines, a trusted care team, and support from loved ones help many find steadier ground and moments of normalcy.
Treatment and drugs
Treatment for Rabson-Mendenhall syndrome focuses on easing symptoms and supporting growth, energy, and day-to-day comfort, since there’s no single cure yet. Care usually includes very high doses of insulin combined with other medicines that make the body’s tissues more sensitive to insulin, careful nutrition planning to steady blood sugar, and close monitoring to prevent complications like dehydration, infections, and dental or skin problems. Some people may receive additional hormone support, medicines that slow carbohydrate absorption, or targeted therapies within clinical trials; your doctor can help weigh the pros and cons of each option. When treatment is tailored to your genes, it’s often called personalized medicine, and genetic counseling can guide family planning and connect relatives to testing. Supportive care can make a real difference in how you feel day to day, and many living with Rabson-Mendenhall syndrome work with a coordinated team—endocrinology, nutrition, dentistry, dermatology, and genetics—to adjust care as needs change.
Non-drug treatment
Living with Rabson-Mendenhall syndrome often means planning meals, monitoring glucose closely, and lining up supportive care to prevent swings in energy and mood. Alongside medicines, non-drug therapies can help steady blood sugars and daily wellbeing. Recognizing early symptoms of Rabson-Mendenhall syndrome can guide timely nutrition, skin, and dental support. Care usually works best with a coordinated team and clear home plans for sick days and school or work.
A dietitian can tailor meals with steady, lower–glycaemic carbohydrates, adequate protein, and healthy fats to smooth glucose ups and downs. Frequent small meals and planned snacks can help prevent both lows and extreme highs. Avoiding long fasting periods is usually important.
Continuous glucose monitoring or frequent finger-stick checks can reveal patterns and provide alerts for highs and lows. Tracking meals, activity, and symptoms alongside readings helps refine the plan. Keep backup supplies and sensors ready.
Gentle, regular movement like walking or cycling can support overall health and insulin sensitivity. Check glucose before and after activity and carry fast-acting carbs to treat lows. Adjust timing of snacks around exercise.
Illness can send glucose and ketones out of range quickly in this condition. A written plan for fluids, extra checks, and when to seek urgent care can reduce risks. Keep urine or blood ketone strips at home.
Your genes may have an impact on how drugs work for you
Even with the same diagnosis, people can respond very differently to medications because gene variants affect how fast drugs are absorbed, broken down, or reach targets. In Rabson–Mendenhall syndrome, extreme insulin resistance from insulin‑receptor gene changes limits insulin’s effect, shaping dose needs and drug choices.
Pharmacological treatments
Medicines for Rabson-Mendenhall syndrome aim to lower very high blood sugar, reduce insulin resistance, and manage complications from extreme insulin shortage. Because early symptoms of Rabson-Mendenhall syndrome can include intense thirst and quick weight loss from high glucose, doctors often combine treatments and adjust them over time. Not everyone responds to the same medication in the same way. Care usually happens in a specialty center with close monitoring for side effects and changes in dosing as needs shift.
This lab-made growth factor can lower blood sugar by acting through pathways that do not rely on the insulin receptor. It’s used by specialists when insulin alone is not working well, with careful dosing to avoid low blood sugar.
Very high doses or continuous infusions may be needed to control severe hyperglycemia. In emergencies like diabetic ketoacidosis, insulin is essential to stabilize blood sugar and ketones.
This insulin-sensitizing medicine can lower liver sugar output and improve how the body uses glucose. It’s often a foundation drug if kidneys are healthy and stomach side effects are manageable.
These medicines help the body respond better to its own insulin over weeks to months. Doctors balance potential benefits with risks like fluid retention or effects on bones.
Genetic influences
In most families, Rabson-Mendenhall syndrome stems from changes in the INSR gene, which helps cells respond to insulin. This follows an autosomal recessive pattern—meaning a child must inherit two changed copies, one from each parent, to develop the condition. A “carrier” means you hold the gene change but may not show symptoms. When both parents are carriers, each pregnancy has a 25% chance of a child with Rabson-Mendenhall syndrome, a 50% chance of a carrier, and a 25% chance of neither. That’s why it can appear even without a known family history, and it may be more common in families where parents share ancestors. If you’re wondering “is Rabson-Mendenhall syndrome inherited?”, genetic testing and counseling can confirm the specific gene change and help guide care and family planning.
Is Rabson-mendenhall syndrome genetic?
Genetics are central in Rabson-Mendenhall syndrome; it results from inherited changes in the insulin receptor gene. Most cases are autosomal recessive, meaning both parents carry a variant. Different variants can influence severity, age at diagnosis, and response to treatments.
Pharmacogenetics—how genetics influence drug effects
Because Rabson-Mendenhall syndrome involves changes in the insulin receptor gene, the body’s response to insulin is limited, and some people benefit more from treatments that work around the receptor, such as insulin-like growth factor-1 (IGF-1) therapy. The exact gene change can influence how severe the insulin resistance is, which in turn affects how much insulin or IGF-1 helps and what doses are needed. When doctors add other medicines, like metformin or a thiazolidinedione, genes that affect liver enzymes and drug transport can shape how quickly the body processes them, the chance of side effects, and the safest starting dose. Genetic testing can sometimes identify how your body handles certain medicines, helping tailor choices and dosing. That said, genetics is only one factor; age, kidney and liver function, nutrition, and other illnesses also shape drug response in Rabson-Mendenhall syndrome. Pharmacogenetic testing in Rabson-Mendenhall syndrome isn’t routine everywhere, but it may be considered in complex cases, especially when side effects or lack of benefit make treatment harder.
Interactions with other conditions
Day to day, intercurrent illnesses matter: if someone with Rabson-Mendenhall syndrome gets a cold or a stomach bug, blood sugar can spike quickly and ketones may rise, making dehydration and fatigue come on fast. Some conditions share “underlying mechanisms,” such as problems with how the body responds to insulin, so issues like polycystic ovary features in girls and women, or fatty liver in some people, can occur alongside this syndrome. Infections, fever, or stress hormones—and medicines like steroids—can further worsen insulin resistance and trigger swings that resemble severe diabetes, including episodes of diabetic ketoacidosis. When sugars run high, skin or gum infections may be harder to clear, and what feel like early symptoms of Rabson-Mendenhall syndrome, such as very frequent urination and intense thirst, can intensify. Over time, high triglycerides and liver fat, if present, may add heart and liver strain, so managing cholesterol and blood pressure becomes part of the broader plan. Doctors call it a “comorbidity” when two conditions occur together, and coordinated care between endocrinology, liver, dental, and reproductive health teams can reduce these knock-on effects.
How genes can cause diseases
Humans have more than 20,000 genes, each carrying out one or a few specific functions in the body. One gene instructs the body to digest lactose from milk, another tells the body how to build strong bones and another prevents the body's cells from beginning to multiply uncontrollably and developing into cancer. As all of these genes combined are the building instructions for our body, a defect in one of these genes can have severe health consequences.
Through decades of genetic research, we know the genetic code of any healthy/functional human gene. We have also identified that in certain positions on a gene, some individuals may have a different genetic letter from the one you have. We call these hotspots "Genetic Variations" or "Variants" in short. In many cases, studies have been able to show that having the genetic letter "G" in the position makes you healthy, but having the letter "A" in the same position disrupts the gene function and causes a disease. Genopedia allows you to view these variants in genes and summarizes all that we know from scientific research, which genetic letters (Genotype) have good or bad consequences on your health or on your traits.
History
Throughout history, people have described children who were unusually small for their age, had patchy darkened skin, and struggled with thirst and frequent urination long before diabetes was understood. Families and communities once noticed patterns that seemed to “run in the family,” with more than one sibling affected, hinting at an inherited cause. In some places, elders recalled relatives who couldn’t gain weight despite eating well, or whose baby teeth arrived early and adult teeth appeared strikingly large.
Early medical notes from the early 20th century gathered these scattered observations into case reports that linked severe insulin resistance with growth and skin changes. From these first observations, clinicians recognized that this cluster of features was distinct from typical childhood diabetes. As insulin therapy became widely used, it became clear that some children needed extraordinarily high doses yet still had very high blood sugar—an important clue that the body wasn’t responding to insulin properly.
By the mid-1900s, case descriptions became more detailed. Doctors recorded consistent features—such as early tooth eruption, thickened nails, and areas of dark, velvety skin—alongside poor growth and metabolic crises. Initially understood only through symptoms, later work started to group these children into recognizable patterns of severe insulin resistance, which included what is now called Rabson-Mendenhall syndrome.
In the late 20th century, advances in genetics confirmed what families had long suspected. Researchers identified changes in the insulin receptor gene as the underlying cause, showing how a faulty “dimmer switch” for insulin signaling could disrupt growth, energy use, and skin and hair development. DNA research now explains why some relatives share milder or more severe signs, and why the condition follows an autosomal recessive pattern—often appearing when both parents carry a silent copy of the gene change.
With each decade, the picture of Rabson-Mendenhall syndrome has grown clearer. Earlier, scattered case reports gave way to careful clinical descriptions, and then to molecular testing that tied the clinical story to a specific gene. Not every early description was complete, yet together they built the foundation of today’s knowledge. This history helps explain why timely recognition matters: noticing the early symptoms of Rabson-Mendenhall syndrome—especially severe insulin resistance in a small child—can guide focused testing, supportive care, and informed counseling for the whole family.