Getting patients well, keeping them well, and focusing on a cure
Do I Have CGD?
After Being Diagnosed
General Information about CGD
Support for Parents/Patients
Doctor Visits & Hospitalizations
Specific Infecting Organisms
Specific Disease Help
Your Time in the Hospital
Updates on CGD Patients (2011)
Updates on CGD Patients (2013)
Professional PID Links
Contact & Nonprofit Info
Doctor Visits & Hospitalizations
Your Medical Records
After each hospitalization, always request in writing:
In some cases (such as a protracted hospitalization), you may wish to omit 2 and 3 above. You should try to give the dates of hospitalization. If you have not requested your medical records in the past, you still should be able to obtain them. Again, it is good to give the approximate dates of each hospitalization. Some hospitals charge a fee for this service, so it is best to check into this beforehand. Each hospitalization record should be kept in a separate folder with the date written on the tab. Please write the CGD Association if you have any suggestions concerning the organization of your medical records.
Child Advocacy – written by Marge Fujikawa
We strongly believe in child advocacy. After many stays in the hospital with our two CGD sons, here are some suggestions for uninitiated parents.
I’d like to communicate some of the things that have helped us deal with the CGD condition of our sons, Patrick and Jason:
Communicate your concerns to the doctors.
You know your own child best. Let them know of your child’s past experiences with any drug reactions. Be sure to tell them of such reactions by close family members, because the information might turn out to be relevant.
Although a sedative, Versed, is used to calm children, it could have, in rare instances, an opposite effect. I mentioned this possibility to the doctors because my older son, Patrick, had previously experienced an energizing effect. Unfortunately, the drug was used on Jason, resulting in a “jazzed up” kid while the PIK line was being put in!
Tell how he copes best and communicate any questions you have.
Write down names of consulting doctors along with their specialties. Weigh their recommendations, knowing their background.
Realize that each of the doctor’s viewpoints is influenced by their own field and experience, and that they may not know how the CGD condition complicates the picture. Jason had five consulting specialists besides his main immunologist, plus many residents, fellows and interns following his condition.
Depending on the staff, they may use inaccurate terminology, gloss over certain relevant facts and generally confuse you. Some residents and surgeons kept referring to the “abscess” in my son’s lung even though the area was just partially walled off. If it had been an abscess, IV medications would not be effective (no blood veins in an abscess), and surgery would have been needed to remove the abscess! It was a bit scary until I calmed down enough to ask the main attending doctor.
Some doctors may have just looked at the medical notes and not realized that X-rays taken on different days had different interpretations. One radiologist looked at the September 10 X-rays, which were nothing to get excited about, and stated that the non-invasive skin tests would be better than a lung biopsy. When asked how familiar he was with CGD, he admitted needing to consult with the immunologist later.
Note your source of information.
Rank the order according to the source of your information. Too many staff are well meaning and try to give you information that may not be accurate or up to date. Rely on your main doctor for progress notes and future planning.
Teach the staff.
Have them note in the files how your child best operates. If he does better knowing details of the procedure, have the staff explain. If your child is good at communicating, ask the staff to leave questions open-ended, not feed ideas to them. Sometimes just asking, “Are you scared?” or “Are you hot?” puts ideas inside the little guy’s head.
Sometimes empathy can be misplaced. If the procedure has to be done (IV, shots, blood work), tell the person not to apologize for doing it. It can also be detrimental for the staff to say, “I don’t like needles either” or otherwise sympathize with the patient on a treatment they have to receive.
Normalize your life. Take control.
Even in the hospital, try to maintain certain normal routines. Get homework from the regular class teacher so your child will not be too far behind in class work. You can also arrange for home visits from tutors sent through arrangement with the public school district office.
Try to gain control of your environment in your hospital room. Decorate the room with your child’s artwork, family pictures and familiar toys. Take a favorite blanket or stuffed animal. Ask what the patient would like from home.
Let your child have control of certain things. Let your child select foods from the menu, decide when to do homework, choose rewards and generally let him have a say whenever possible.
Keep things matter of fact.
Chronic conditions can make planning ahead a bit difficult. Experience life one day at a time. Appreciate what you have. Try not to project too far into the future since the scenarios can change. Be realistic and know the limitations placed by CGD, but also explore alternatives.
Let your child express himself.
Your child may be suppressing fears and anxieties.
A Child Life Support staff can demonstrate procedures using puppets and dolls. Your child can ask questions about anything that may happen in the hospital.
Let your child draw/paint/write about the hospital experience. Have them keep a journal.
Let your child take out anger at you, the parent. You are considered safe.
Choose coping skills and enjoy life.
Use support systems. Talk to other parents with kids with chronic conditions.
Take breaks. Have other people watch your kids. Trade off.
Get a change in scenery. Drive off into the sunset. Go camping. Take a 3-day weekend.
Pain Management – written by Lonnie Zeltzer, M.D. UCLA Pediatric Pain Program
I appreciate the opportunity to discuss pain management, since I believe that both child and adult patients and their families can act as advocates for good pain control if they know what they can expect. I will review the psychological and pharmacologic strategies that are used to treat pain. However, there are some facts about pain that need to be discussed first. It is important to realize that the amount of "pain" is not simply related to the amount of tissue injury. There are many other factors that contribute to the pain experience. For example, you could feel the sensation of an injection (sensory aspect of pain) but not be bothered by it (affective or emotional aspect of pain); conversely, at another time when you might be sad, angry or worried, that very same injection might bother you a great deal. The reason that the same potentially painful experiences affect people differently or might affect you differently at different times is that your "pain inhibitory" (pain control) system is always working whenever there is stimulation of the nerves that transmit "pain" signals (e.g. during injections; chest tube placements, etc.). This information is critical to know because it means that there are things that you can do to disrupt the passage of the pain signals to the brain (ascending pain control) or to change the "messages" once they get to the brain ("J descending" pain control). I will review the various ways that you can modify your own pain experiences and the types of drug treatments for pain that you can discuss with your doctor. It is important to tell your doctor about your pain so that it can be treated properly.
Self-Regulation of Pain
Feelings, attention, memory, beliefs, expectations, and simultaneous physical stimulation all affect the pain experience. Melzack and Wall, scientists who developed the "gate control" theory of pain transmission/inhibition, found that stimulation of certain nerves (e.g. nerves for temperature, pressure, other sensations) at the same time that pain nerves were sending signals (e.g. during an injection) blocked the movement of these pain signals up the spinal cord so that they would not reach the brain to be translated as "pain". They were "gated" so to speak somewhere in the spinal cord in order to allow the signals from the larger sensory nerves to be transmitted (e.g. like cars being stopped at a drawbridge to allow a large ship to pass). If the pain signals do not reach the brain, pain will not be experienced. Thus, other physical stimulation (e.g. rubbing or pressing a part of the body near the injection site) can reduce or stop the pain signals from reaching the brain.
There is also a "descending" component of the pain control system that "gates" or alters the signals within the brain. Once pain signals do reach the brain, however, many things have to happen in order for the brain to "translate" these signals to be interpreted as the pain that you then experience. First, your mind must interpret these sensations as pain (something that you may think is out of your control and will bother you, something to fear) in order for you to feel these sensations as pain. If you have had bad past pain experiences, you may interpret similar physical sensations as pain and may expect pain when you see anything related to those earlier experiences (e.g. the needle). Thus, the memory of past pain may cause certain chemicals (stress hormones) in your body to increase and you might feel their effects as anxiety (fast heart beat, faster breathing, sweating, etc.). These chemicals will then increase the sensation of pain and the amount it will bother you. Attention on the pain sensation can also increase the pain.
Thus, to alter the pain signal, you can do the following: 1) keep your attention on something else, such as imagining being somewhere else or doing something else, concentrating on a math problem or a song, or being engaged in a conversation or listening to or telling a story; 2) change your expectations about pain, since each pain experience can be different; 3) concentrate on non-painful aspects of the sensory experience, such as pressure, tingling, etc.; and 4) take slow, deep breaths, while concentrating on your breathing, in order to relax your body. As you experiment with different ways of coping with pain, you will learn what works best for you. It is important to remember that you are able to change your pain experiences so that they can be more under your control and you can learn ways of increasing the effectiveness of your body's natural pain control mechanisms.
Drug Treatments for Pain
There are a variety of drugs that can be used for acute pain (e.g. medical procedures), persistent but short-term pain (e.g. post-surgery), and chronic pain (e.g. arthritis, other disease-related pain). These should always be discussed with you or your child's physician so that you can plan together for good pain management, just as you do for control of infections. Drug treatment of pain can always be used in combination with psychological methods of pain control.
For medical procedures, local anesthetics should be allowed 5-10 minutes to "work" before the procedure is started. If the patient is extremely anxious before a planned procedure, a drug like valium taken one hour before the procedure or liquid versed 20 minutes before can reduce anxiety and thus pain. During the medical procedure, there are a number of options, if drugs are to be used. For example, if an IV line can be easily placed, a combination of IV versed and fentanyl can be used if the physician has experience with these drugs and the appropriate equipment to monitor oxygen (pulse oximetry on the finger tip), heart rate, and blood pressure is available. There are other drugs, such as nitrous oxide, ketamine, and others, that may require the presence of an anesthesiologist or pain specialist. Intramuscular injections for pain control should no longer be needed because of newer methods and drugs. For postsurgical pain, PCA (patient controlled analgesia) is a newer, more effective way of giving narcotics for control of persistent pain. PCA requires a computerized machine in which the drug delivery method and doses are programmed into a computer, so that a continuous amount of drug can be delivered to the patient (e.g. at night), a pre-set amount of drug can be delivered whenever the patient pushes a button (when more is needed), and there is a "lock-out' period programmed so that the patient will not get more drug than is safe within a given time period. For chronic pain, drugs such as Tylenol and ibuprophen (motrin, advil) can be used for mild pain, and various types of narcotics for moderate to severe pain. Taking narcotics for pain will not produce drug addiction and is not harmful if prescribed appropriately. There are also other drugs that add to pain control, even though they are not direct analgesics. Pain management should always be discussed with your doctor, so that advanced planning can reduce the likelihood of pain problems later.
[Ed. Note: We are grateful that Dr. Lonnie Zeltzer has taken the time out of her busy schedule to write about pain management.
Fear of Shots – written by Lonnie Zeltzer, M.D. UCLA Pediatric Pain Program
Why Children Have a Fear of Shots
Many children fear "shots", such as the injection of gamma interferon, for a number of reasons. The shots may have hurt a lot, and children may feel that they have no way of controlling the pain. Thus, they worry that they will become overwhelmed by it. They may also be "pain sensitive" so that they feel pain more readily than others. Memories of bad experiences frequently influence expectations of future pain, cause fear before the shot, and reduce the child's belief that he can control both the fear and pain experience.
Fear and Pain with Shots Can Change
As noted in the previous issue of the Newsletter, the transmission and control of pain signals, such as those from a shot, are part of a flexible system. Memories, beliefs, expectations, fear, attention, and feelings of control over the pain all contribute to the experience of pain. Thus, the pain experience can be different with each shot, even if the shots are given in the identical way.
What does this mean for children getting shots repeatedly?
What You Can Do to Make Shots Tolerable
Role of Parents
Parents of children who have resisted shots should first learn from their child more about the child's particular experience with shots. Parents should not assume that their child's feelings about shots are the same as their own. They should try to get to know, for example, what aspect of it bothers their child the most: anticipating it, the needle going in, the injection of medication, etc.
Parents should try to find out what their child does before, during, and after the shot. In "getting ready", does he start worrying that it is going to hurt? What thoughts go through his mind? Does he feel like he will not be able to control himself? What does their child do to cope with the fear and/or pain during the shot? Does he think about other things or does he concentrate on the shot or pain? Finally, how does he feel after the shot? Is he angry, sad, shameful, or scared? Information from these questions plus knowledge of the child's responses in other pain situations, such as stubbing a toe, falling off a bike, and the like will help parents to find the best ways of helping their child.
If anticipatory anxiety (fear before the shot) is a major problem, the parent can tell the child that "it can go better ...and we can do some different things together to see what works best." Some children "catastrophize", that is to say they cannot get the thought out of their head that it will hurt and, the more they think about the pain, the more scared and out of control they get. If catastrophizing is a problem, parents can teach their children to "thought-stop". For example, the child can learn to say "Stop! I won't think about the shot anymore! I know what I can do to get through it." This technique must be used in conjunction with methods that the child can use during the shot so he can cope with it. Parents can guide their children to the technique from which their child will most benefit.
These methods include helping the child to:
Role of the Child/Adolescent
Children and adolescents can learn and practice all of the above methods (e.g. they can give themselves a "shot" with a syringe without a needle while using the above methods). Children will learn quickly what works best for them. For variety and interest, children may want to change methods over time or "invent" their own. They might want to try picturing different sensations, such as hot, cold, pinching, etc. during the shot. Younger children may prefer to blow bubbles with a soapy bubble-maker. The goal is for the child/adolescent to feel that he has the ability to "keep a lid" on his fears and to cope with any discomfort associated with the shot.
Positive experiences of coping with shots, in turn, lead to increased feelings of effectiveness or abilities to cope. These, in turn, reduce the associated distress. Children and adolescents who learn to cope effectively with shots can also apply this skill to other pain situations. Mastery over these pain situations can lead to increased feeling of self-control and self-pride, both of which should be reinforced through praise by parents.
After the shot, it is helpful to discuss with the child what worked best and plan together what to do to make it even better. Such planning helps the child to begin to think about his own strategies for coping. Praises should always be given in order to let the child know how brave he was to "get through it", regardless of whether or not the method he chose to use was effective.
[Ed. Note: A big THANK YOU to Dr. Lonnie Zeltzer for writing "Fear of Shots". This article, as well as her previous article, "Pain Management" will be kept in a permanent file to help CGD patients deal with pain.
Anesthetic Cream to Take the Pain and Fear out of Shots
The latest issue of the Ways & Means newsletter, published by Quantum Health Resources, Orange, CA., mentions a new anesthetic cream, EMLA, that can take the “ouch” out of needle sticks. The cream is easy to use, but for it to be totally effective, it must be applied one hour prior to the needle procedure. During this period, the cream is protected by a patch so that the patient can move about freely during the waiting period. ASTRA in Westborough, MA 01581, is the manufacturer of EMLA. It is available only by prescription.
There are CGD patients who have considerable fear of their interferon gamma injections. You may wish to discuss this procedure with your treating physician. If you or your child has CGD and you have used EMLA or a similar cream or device that has helped ease the pain or fear of needle sticks, please write the CGD Association so that information can be shared.
To Share: Inject-Ease and Fear of Shots – written by Nancy Finkell
Kids (or anyone) having trouble sticking a needle into themselves (or others) while taking injections of gamma interferon, might be interested in trying Genentech’s “Inject-Ease.” It’s a plastic contraption that holds the syringe and when placed against the site, the mere push of a button quickly and painlessly releases the needle. The medicine is simply pushed in the usual way. It’s perfect for camp and overnight trips because ANYONE can do it! Some pharmacies carry the Inject-Ease, but it also can be ordered through the vendor of your interferon.