Caregivers' Guide: Section 2

Beginnings
One day, when we were having a particularly traumatic time in the pediatric intensive care unit, I found a phone and called my pediatrician. He was on the phone instantly and talked to me for fifteen minutes, interpreting for me the jargon being used by the specialists and giving me some guidelines about how to approach them with my concerns. With his help, I went back in feeling more confident, not like some intruder in a world I didn’t understand.

Dealing With Doctors
If you have never spent a lot of time with doctors, now is the time to make up for it. Since ichthyosis is a rare and complex condition, many specialists will probably be brought in on your baby’s behalf. Although the number of professionals may intimidate you, as you become familiar with each of them and their functions, you will begin seeing them as your baby’s team.  Remember, you and your physicians are on the same side; you both want what is best for your child.

Get a notebook that you can easily carry in the hospital and begin making a record of the professionals you meet. Note their departments, specialties, and how to reach each of them. Use your notebook to keep track of tests, medications, your baby’s progress, and your doctors’ observations. Write down unfamiliar words you encounter and don’t be reluctant to ask a doctor or nurse to stop and spell a word and explain its meaning. Don’t hesitate to ask questions or express concerns you might have to any of your child’s caregivers. Early on you will discover that you relate more to some doctors or nurses than others; these few can serve as helpful resources and friends in the neonatal intensive care unit’s confusing environment of machines and medical terminology.

Find out right away who is in charge of your baby’s care. This may be a neonatologist, a physician who specializes in the treatment of newborns. In the case of infants with ichthyosis, however, primary care may fall to a dermatologist or pediatric dermatologist. If your baby is at a teaching hospital, there may be several levels of authority and responsibility. This can be confusing, so make certain you get a clear picture of these lines of responsibility.

If your baby’s doctor is on a tight schedule and doesn’t offer you the time you feel you need, or if you want time to organize your thoughts and questions, set up an appointment to meet him or her in the privacy of an office. If, understandably, you are having trouble expressing yourself clearly or retaining information, consider bringing along a friend or family member with a clear head. If you can, also bring a tape recorder so you can replay the information later to refresh your memory.

It is easy to feel intimidated by doctors. Don’t be! Most doctors appreciate parents who express a willingness to be informed about their child’s condition. Remember that your doctor has other patients and parents, and respect his or her time by organizing and prioritizing your questions. But be assured that you have rights, too.

You and your partner are your child’s most important advocates. You are entitled to any information you seek.  If you don’t understand something your doctor says, ask for a fuller explanation. If you don’t understand why your child is receiving a particular treatment, ask. Don’t be afraid that a question might make you look ignorant and don’t be afraid to speak up! It is vital for you as a parent to feel in control of the well being of your child.


Feeling at home in a Neo-natal Intensive Care Unit

Our daughter spent the first month of her life in a neonatal ward. My husband and I made her space as warm and personal as possible. We taped family pictures to her isolette and brought a small tape recorder and some soothing tapes from home. Our daughter particularly seemed to enjoy a tape of ocean sounds so her nurses, getting into the spirit of things, made her a colorful mobile with palm trees and sailboats. I think all of these special touches comforted my husband and me as much as it did our baby.

When you first walk into a neonatal intensive care ward with its high-tech equipment and imposing staff, it can be a frightening, intimidating experience. However, by the time your baby is discharged, it will probably feel like a second home.

Familiarize yourself with the ward, its rules, and staff. Get acquainted with the equipment and the function of each piece. Don’t be afraid to ask questions. Though many neonatal wards are relatively crowded, don’t feel as though you’re in the way. This is your baby’s temporary home, so pull up a chair and relax. Also, get to know your baby’s nurses; this way, when you are at home, you can feel comfortable about who is caring for your baby.  The nurses will be your first source of information about how your baby is doing, since they spend the most time with him or her.  You can request that the same one or two nurses care for your baby every day.  Having the same caregivers every day will allow the nurses to learn as much as possible about ichthyosis, its special circumstances, and how it affects your child. Remember, your baby is an infant with the need to sleep many hours.  You have the right to limit the medical professionals who see your child to those who are immediately necessary.  You have the right to ask a doctor or technician to come back at a time when the baby is not sleeping.

One family worked with the nursing staff to set up two cribs for the baby.  One crib was decorated with comfort items and pleasant stimuli.  This was her safe place; nothing painful could happen here.  The other crib, on the other side of the room, had only white sheets.  This is where dressing changes, baths, blood work, and other procedures took place.  The staff purchased a brightly colored apron that they all wore during the painful procedures.  This was a trigger that the painful part was about to happen.  The baby realized the difference and would recover quickly when placed back in her safe crib.

It is extremely important that you plug into your baby’s care cycle. This will offer a valuable bonding opportunity as well as help you prepare for your baby when he or she comes home. Help the nurses give baths and put on lotions and ointments. Taking part in your baby’s care will also build your self-confidence and increase your skill at managing your baby’s special needs. It will provide the “hands on” experience you will need as you explore doctors and treatment options for the future. Despite many parents’ initial instinct to stand on the sidelines and leave things to the “experts,” now is the time to become active and learn everything you can about your baby and his or her skin. Remember, your baby is coming home soon, and then you will be in charge. You must have the skills by this time to take over his or her care.

Remember, too, that these first weeks are a time for a mother to rebuild her own strength after the rigors of delivery. You cannot spend all of your time with your baby at the hospital, so don’t feel that you should. Don’t feel uneasy or guilty about taking time for yourself and the rest of your family. Don’t let yourself feel guilty when you are not at the hospital with your child. It helps no one if you allow yourself to become physically and emotionally drained. 


Bonding with your baby
After we were finally allowed to hold baby Joshua in sterile blankets and a cap, ourselves outfitted in sterile gloves and gowns, we could not resist the joy of kissing his forehead when no one was looking.

A baby with ichthyosis can be a very different looking baby. In some cases, your child may look frightening. Even parents with “normal” babies often take weeks before they feel especially close to their infants. It is only natural that you might feel detached and even afraid to hold your baby.

Take a deep breath, relax, and give yourself time. Initial rejection is not abnormal, and it can be worked through. You must allow yourself, your spouse, and your family time to grow close to your new baby. Often, before this can happen, you must cast aside your preconception of the “perfect” baby and get to know the baby you gave birth to. You must put your fear aside and look beyond your baby’s unusual skin. Remember that beneath the skin is a normal infant who needs to be loved. Hold your baby as soon as you can. Hugging, kissing, and stroking your baby will help you bond. Almost at once you will begin discovering your baby’s special qualities. All babies have them.

If for some reason you are told not to handle your baby (some types of ichthyosis prohibit immediate handling), or if you just don’t feel comfortable with the idea at first, do what you can to let your baby know you are there. Hold your baby’s hand, stroke his or her head, read a story or sing a song. Your baby is one of the few people who will never get tired of hearing your voice. In fact, your voice is so soothing to your baby, consider taping yourself reading or singing to comfort him or her while you are away.


Breast feeding your baby

The first time I ever really felt maternal toward my baby was the day when I could finally turn my back on the bright lights of the neonatal ward, cuddle her on a mountain of pillows and nurse. At last, I felt like she needed me and I was giving her something neither the specialists nor the machines could provide.

Breast feeding should not be ruled out because your child has ichthyosis or because he or she is in the hospital. Mother’s milk is the most nutritionally sound food for your baby. It promotes optimum health because it provides specific immunities against illness, and proteins that enhance development. Since children with ichthyosis are often susceptible to infection, breast feeding should be seriously considered when evaluating your baby’s feeding needs. Breast feeding also prevents over feeding and offers emotional rewards to both you and your baby. It can help you overcome the feeling of separation you may feel while your baby is in the hospital.

A baby born with a collodion membrane is often capable of breast feeding. In fact, the area around your baby’s mouth is often the first place the membrane begins to shed. If your baby is in an isolette, don’t feel intimidated. Cream your baby up and put him or her to the breast. If you and your baby have difficulty getting a good latch, don’t give up. Patience is important, for nursing comes naturally only to a lucky few; most moms and babies have to work at it for a while. Ask an experienced nurse for help, and if you continue to have trouble, consider seeking the help of a lactation specialist. Most hospitals will have one on staff who can help you, and if not, your doctor or nurse can recommend one. You can also contact your local LaLeche League.

Some babies, especially those with EI, need to be handled gently because of the fragility of their skin. If the fragility of your baby’s skin makes nursing difficult, electric breast pumps are available for collecting milk for later feeding. Regular pumping will also build up your milk supply for the day you are finally able to nurse. Sometimes the stress of having your baby in the hospital will prevent you from nursing effectively. In these cases, too, using a breast pump and bringing mother’s milk to the hospital will help your baby enormously. Then, when your baby does come home, it may not be too late to begin nursing him or her.

If you can’t or don’t wish to nurse, commercial formulas provide perfectly fine nourishment for your baby. Millions of healthy babies have been raised with the bottle, and yours will do just fine. However, consult your physician about nutrition supplements, such as iron, that may be helpful.


Doctors for Longterm Care
When your baby was born, you acquired a group of doctors whom you did not choose. Contact with these physicians gave you a good chance to evaluate specialists that you might turn to for long-term care for your child. At the same time, you are not bound to return to anyone for follow-up visits if your experience with them in the hospital was negative. You may turn instead to other specialists in your community.

As you evaluate specialists, keep in mind that most types of ichthyosis are very rare. You want doctors who have not only an academic, scholarly interest in medicine, but also clinical experience and a special interest in treating ichthyosis. These are the physicians who monitor medical journals, textbooks, scientific meetings, and pharmaceutical data to ensure that your child is benefiting from the latest and best research. Most important of all, you must feel that you can build a long-term professional relationship with the physician, one in which you are working together to manage a common problem.

To find such a physician, contact FIRST and ask for a physician referral in your area.  FIRST maintains a list of physicians who are knowledgeable about ichthyosis, are treating other patients with ichthyosis, or who have an interest in seeing patients with ichthyosis. Or, you may want to contact an accredited medical school in your area for a list of names of faculty members who have dermatology practices in your community. A pediatric dermatologist is often a good choice because these sub specialists are used to caring for infants and children with skin diseases. At the very least, find a dermatologist with an interest in ichthyosis.  A doctor who might be fine for teenagers with acne, or one who is solely a skin cancer specialist, may or may not have the professional curiosity and motivation necessary to monitor a complex disease in an infant.

You will also need to choose a pediatrician very carefully. For many children with ichthyosis, additional time and attention to skin care will be the only medical issues that set them apart from other children.  However, other issues may be a problem for some children.  Minor childhood diseases like chicken pox can be serious in a child with ichthyosis. Some children with ichthyosis do not sweat properly and can overheat dangerously.  Some children with ichthyosis cannot wear adhesive bandages because removing them tears the skin. Nutrition and growth can also be a significant secondary concern for your pediatrician and dermatologist to watch carefully.

For most children with ichthyosis, physical, intellectual, and psychological development can be perfectly normal.  Some forms of ichthyosis are associated with developmental delays, and in all forms of ichthyosis thickened skin blunts the touch sensation.  Physical (motor and fine motor) and cognitive development, vision and eye control, and psychological well-being can all be affected by ichthyosis and require the support of a physician who is scholarly, yet practical, kind and compassionate without being maudlin, and accessible to you when you need advice. Federal and state funding is often available to help cover the costs of such things as delayed motor development, and a doctor can assist you in obtaining such aid. He or she can also be your advocate with insurance companies who are unfamiliar with the condition and therefore deny claims related to your child’s disorder.

If you are in an HMO or PPO that requires you to go to a certain clinic or panel of doctors, it is worthwhile scheduling an appointment with an administrator who can help direct you to one physician who will be responsible for your child’s overall care. This is to everyone’s advantage, since it will avoid having to explain ichthyosis to each new physician you encounter.

It is a good idea to encourage professional relationships between physicians caring for your child. A good dermatologist/pediatrician team will work together on such issues as diet, skin infections, chicken pox, stitches, casts, and other medical concerns that cross specialties where ichthyosis is concerned.

Many parents have found it useful to seek a consultation with an expert in ichthyosis at least once (FIRST can provide you with the names and locations of many of these physicians), even if they have to travel some distance and pay for it out of pocket. This evaluation can serve as a way to make sure that your child’s regular physicians are providing state-of-the-art care, and can provide someone to call for a second opinion when future medical decisions must be made.

As your child gets older, talk to him or her about the doctors you have selected. Do they like them? Do they feel comfortable with them? (One mother found that the doctor her three-year-old liked best was the one who provided a cotton child-sized examining gown so she did not feel on display during examinations.) Many teenagers with ichthyosis have expressed a desire that doctors talk to them and not their parents. If your child has a problem with a doctor, discuss it with the physician and consider changing to someone who will lessen your child’s anxiety.

Interested doctors and nurses can offer tremendous support. Dermatology nurses, for example, can demonstrate effective ways to apply medications and dressings. Pediatric nurses often have great practical ideas for distracting a child during painful treatments. Nurses can interpret medical terms for you, help you get a doctor on the phone, and give you samples of medications they know you use. Seek them out as allies.

Once you have addressed your child’s medical needs, you may want to consider choosing a mental health professional to help you through the traumatic early months of parenting a child with special needs, and to follow your family in the future. Depression can be a problem for people with ichthyosis, and may require intervention by a professional. But less serious psychological issues deserve airing, too. Some parents find it helpful, especially for pre-teens and teenagers, to have a child or adolescent psychologist, or social worker, work through the emotional aspects of what can be a disfiguring condition. It is useful for adolescents to have this kind of support from someone who is not a family member. Seek references from a trusted physician, a children’s hospital, a nearby university hospital, or a state social services agency.


The Finances of Major Medical Events

When all of the doctor and hospital bills started rolling in, my first response was to panic. My wife and I had set aside money for an average delivery and that was all. I forced myself to push all my worries to the back of my mind and focus all my energy on our new baby. When we knew she was out of the woods, I started dealing with the hospital financial department and our insurance company. I was able, in most cases, to set up long term payment plans that fit into our budget.

Children born with ichthyosis usually spend some time in the hospital, anywhere from a few days to a month or more. Since hospitalization will likely be in a neonatal intensive care unit, the costs can be astronomical.  Additionally, throughout infancy and childhood, your child will probably require at least occasional, if not frequent, specialized medical attention. In short, the economics of ichthyosis can become a significant factor in any parent’s life.

The first rule of the economics of ichthyosis is don’t worry about it. In the beginning, put financial worries on the back burner. Instead, focus on your baby and his or her condition. Focus on your family. Learn about ichthyosis and give your attention to getting your child home, where you can create a warm, secure environment.

If you have medical insurance
If you have medical insurance, review your policy. If you don’t have your most recent policy handbook, get one. Once you are familiar with your coverage and benefits, schedule an appointment with the person at your place of employment who oversees your company’s health care insurance plan to discuss your situation. Be prepared to discuss ichthyosis, your child’s prognosis, and the long-term care commitments you may be facing. However, don’t try to predict the future just to answer a question. Where unknowns exist (and there will be plenty of unknowns), make clear to policy administrators and insurance claims adjustors that some questions cannot be answered.

If possible, obtain a copy of your company’s policy contract. The fact is, your health insurance carrier is probably unfamiliar with ichthyosis. In the course of filing claims, they will be evaluating unfamiliar medical circumstances to make reimbursement determinations. These determinations frequently will be made on speculative grounds.

You may find that your insurance company occasionally (and perhaps frequently) does not allow coverage or reimbursement for various procedures and/or treatments. You always have the right to appeal any and all determinations. Your first line of defense against denial of benefits is being fully informed. If necessary, enlist the assistance of your child’s primary physician to support your appeal. Discuss problems with your health insurance administrator at work. If you must, see an attorney. Experience has shown that appeals work.
 

After your child is born, any change in employment that changes your insurance coverage may bring into play pre-existing condition exclusions. Some insurance companies deny coverage to those who need it most by refusing to insure anyone who has a pre-existing condition; that is, an illness or medical condition that existed prior to the application for coverage. This may apply to any genetic disorder, including ichthyosis. However with the newly initiated Affordable Care Act, pre-existing conditions are less of a concern. You will find more information regarding enrollment at www.healthcare.gov.   Or you can access your state’s government website by using www.state.(your state).us., i.e. www.state.pa.us.

If you do not have health insurance
If you do not have health insurance, don’t despair. Your baby will not be denied medical care. However, studies show that the uninsured receive fewer medical services and generally lower quality of care than people who are insured. In order to ensure your child’s long-term health-care needs, you may need to take advantage of one or more state and federal assistance programs. Because assistance programs, as well as criteria to qualify for them, vary greatly from state to state, we cannot offer a blanket summary of programs. However, a few things are essential.

Make an appointment with a social worker at the hospital in which your child is being treated (or in your state of residence, if it is different from the location of the hospital). If no social worker is available there, contact your county or state Department of Health and Human Services. The department will be able to tell you where to go to learn about programs available to you and your child. Regardless of other programs available in your state, one program is key: Supplemental Security Income (SSI), which is under the Social Security Administration (SSA). Federally funded and state managed, SSI provides for medically needy people of all ages who meet the criteria for medical and financial need. Perhaps the most important advantage of becoming eligible for SSI benefits is that in most states this will qualify your child for Medicaid, which is essentially state-paid medical insurance.

Experience has shown that SSI/Medicaid coverage for children with ichthyosis is often denied upon initial application. However, coverage is frequently granted upon appeal. The program allows for several levels of appeal. Because coverage, once granted, becomes retroactive to the date of your first application for the program, it is important to begin the process as soon as possible. The process is frequently long and frustrating, but the benefits of coverage far outweigh the difficulties of the application and appeal. You will find more information with regard to applying for SSI/Medicaid coverage at www.ssa.gov.


This information is provided as a service to patients and parents of patients who have ichthyosis.  It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents.  Neither FIRST, its Board of Directors, Medical & Scientific Advisory Board, Board of Medical Editors, nor Foundation staff and officials endorse any treatments or products reported here.  All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder.


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