Adherence Strategies: Q&A with Dr. Richard Ward
February 13, 2014 -The Cooley’s Anemia Foundation often gets questions and comments concerning strategies for remaining adherent to thalassemia treatments. We have asked Dr. Richard Ward of Toronto General Hospital to respond to some of these questions. CAF thanks Dr. Ward for taking the time to share his knowledge and experience with us and with the thalassemia community.
General comments on adherence in thalassemia:
Dr. Ward: Other patients and families are often a great resource for learning the tricks of the trade with respect to adherence strategies. As individuals who have lived through what you or your child may be experiencing, their advice is usually the most salient to you. If you live away from a large center and don’t have access to peer support, please contact the Cooley’s Anemia Foundation office (Eileen.s@thalassemia.org; 212-279-8090 x 205) to find out about resources that you may find useful, including reputable online forums.
When considering ways to improve your adherence, the key step is to set a realistic goal for what you believe you can commit to and achieve at the present time. As circumstances, and life, changes, these expectations of yourself or your loved one may also differ. What used to work may no longer be suitable, especially as a child grows and develops a sense of autonomy and later a blossoming social life, and eventually an adult life of their own.
I always suggest making a therapeutic contract that you can pledge to keep, and hold yourself accountable to. It doesn’t necessarily have to be something you share with others, but it is more effective if you put it down in writing, as this makes it less theoretical and more tangible. You must also have faith in the chelation drug you are using as it’s been shown to impact adherence. If you’ve lost faith in the ability of the drug you are using, it may be time to discuss switching to another medication.
You may find it hard to be open and forthcoming with your nurse or doctor about how you are finding your medication. This may be due to feelings of shame, not wanting to disappoint them, or perhaps fear of being lectured at. Please bear in mind it is very difficult for your medical team to provide the care you need if they are not fully informed of what’s going on. They will never be angry or upset, but will try with a sympathetic ear to understand the challenges you face and provide tools and counselling to help you succeed.
There can be many barriers to chelation adherence, including: financial barriers to medical care, cost of medication, inadequate follow-up in place, absence of family support, and feelings of depression. Finally, for some there is simply a psychological block that is preventing the individual from reaching their ideal medication adherence consistently. In such cases, the assistance of a social worker or psychologist can be useful, providing cognitive behavioral therapy to overcome the roadblocks.
In summary: the first step is to recognize there is an adherence problem, next identify what it is and what may be causing it. Then decide on a reasonable goal to set in a realistic timeframe. Take small steps so that you can succeed, and take it from there!
Remember, you’re not in this alone, and don’t forget to congratulate yourself when things are going well!
1. What can I do to make taking Exjade easier for my child? Both in terms of the taste and texture and in terms of occasional nausea?
Whether you are a child or an adult, Exjade (deferasirox) can be difficult to swallow. The initial studies with Exjade were by taking it in the morning on an empty stomach. However, it has been shown that it is safe to take with or after food, and at any time of day. In fact, the amount of drug absorbed into the body may even be increased if taken this way. Many patients find that taking it with meals reduces the stomach upset.
Taking it in the evening can also be less disruptive to your routine as you (hopefully) sleep through any side effects.
Patients should experiment with various juices to see what they find easiest to mix the Exjade in. Some find the mixer cup provided by Novartis gives a more consistent feel to the drink and more palatable, less chalky. Another trick is to mix the Exjade in the evening and then drink it in the morning, having given it a new stir beforehand. This can also reduce the chalkiness.
If you are lactose intolerant, the use of Lactaid supplements may relieve some symptoms, as the drug does contain lactose.
Finally, if the dose is too high for you in one go, another option is to take half the dose in the morning and the other half at night. If there are problems with a feeling of sickness at the thought of taking the medication, termed “anticipatory nausea,” then anti-nausea medication may be required, and can be effective.
2. Do you have any suggestions on how to make using Desferal easier for my child/myself?
As someone who doesn’t have thalassemia, I imagine this has got to be one of the hardest aspects of living with the condition. At the end of the day, it may simply require some bribery and positive reinforcement for your child! Fortunately, with oral chelators more readily available over the past decade, the need for Desferal (deferoxamine) has reduced dramatically.
The first question to consider is whether you can make the switch to oral medication. (In particular, subcutaneous intermittent chelation is not ideal, as it leaves you unprotected from the iron during the time that the Desferal is not infusing.)
There are a few things to consider as to why you may be struggling:
- willingness of a child to take the drug;
- needle phobia or pain;
- painful injection sites or reactions;
- inconvenience of the pump.
There are many different types of needles available and if the current one is causing discomfort, it may be worth changing the size (width or length) or the design of the needle (butterfly, thumb tack).
Are you using a local anesthetic spray or cream to numb the area before injecting, and if so, are you leaving enough time between application and injection? If the infusion sites are painful, are they being rotated often enough to allow time to heal? Is the concentration of the Desferal too high? Is there a Desferal skin reaction? (This may be severe enough to require referral to an allergist for desensitization.) Have you spoken to your pharmacist about adding a small amount of steroid to the infusion? Do you need to lengthen the infusion time?
Just as there are a variety of needles, there is also a wide range of different pumps. It may be that the pump is noisy if battery powered and disturbs you or your child at nighttime. Is it shaped such that it is inconvenient to carry? If this is the problem, you should discuss with your nurse the different pump options.
The one nice aspect of using Desferal is the positive, visual reinforcement you get from seeing the urine change color and become red as the iron is excreted by the kidneys. If your child is a budding artist, this could be utilized by recording the number of days of color change each month.
3. How can I encourage better adherence for my child without coming across as nagging (which he won’t respond to)?
This is really tough, and the strategies will need to change as your child matures. Many of these tricks are applicable to many general life situations outside of thalassemia. I would therefore encourage attendance at any education sessions in your area that may deal with child-parent relationships.
As the adolescent brain can develop behind the rest of the body, this is a challenging time of life for patient and parents. However, as an adult hematologist I see first-hand that some youth really respond to the change of environment from a pediatric to adult healthcare setting. Acknowledging your child’s growing autonomy and independence of thought, although hard, will yield positive results for many. Remember, once they hit adulthood, your ability to nag or enforce decisions disappears.
Teamwork and setting jointly agreed upon and mutually acceptable goals is going to help with achieving success. You must take into account your child’s school demands as well as social life needs, which are also very important to their successful transformation into a well-rounded adult.
Above all else, it is vital to not make this the defining aspect of the parent-child relationship as you want your child to grow up to be an individual who happens to have thalassemia rather than a “thalassemic” or “thal patient.” Try to keep a non-judgmental, open dialogue open so they do not feel the need to hide their adherence issues from you. If your child knows they can have a frank discussion with you about their chelation struggles, that’s half the battle won. You will then be able to demonstrate understanding and help them use life skills and coping mechanisms to overcome the current hurdles. These are skills you can teach them, thereby being a partner rather than boss in the relationship.
As your child gets older and learns more about their thalassemia, the opportunity exists to engage them in the decision making process. This may be looking at their ferritin or MRI results and explaining this. Nowadays, many clinic have electronic health records with graphs showing results trending over time. It is also often possible to actually look at the MRI images and see the darkness or light with more or less iron in the organs. For visual learners, these methods can be impactful.
When a little older, your child can begin to record their results and progress. Keeping a journal can be helpful in working through their own struggles, as is being part of a peer support group. Finally, as your child is more independent, let them lead the discussion with the healthcare team, encourage them to ask questions, and even let them see the nurse or doctor whilst you stay outside the room.
Unfortunately, at some point in childhood or adolescence most patients have a period of struggling with chelation. It’s important to realize that the vast majority do come out the other end absolutely fine. Thanks to MRI scans and more chelation choices, even those who have a bad time of things can usually be fully recovered afterwards.
4. I’ve been chelating for years. I know it’s important, but sometimes I just don’t do it. What can I do to motivate myself?
Firstly, be honest with yourself and your healthcare team, and secondly, set yourself up to succeed! If you are not truthful to your own self you won’t ever be able to make the changes necessary to have consistent adherence. Likewise, if your nurse or doctor is not informed of your difficulties they will be unable to assist, and may even make treatment recommendations that are detrimental. It is very rare for your nurse or doctor to get upset. They usually have an inkling of what’s going on and are waiting to lend a sympathetic ear and help you. If they do get angry, you may need to find a new provider!
With a chronic illness it’s really important to maintain the motivation long-term. That’s why making small incremental changes is a good way to approach things. You should commit to a small change and then hold yourself accountable. As you (hopefully) achieve it, you’ll be more motivated to make further changes. For example, if you are prescribed 2000mg of Exjade daily, but are only really taking 1000mg and 3 days a week. Decide what you can achieve in the time to your next transfusion appointment. It may be to take 1000mg, but to do it 5 days a week. If you then achieve that, you can work to a new goal, and thereby make major progress over time to reach the optimal results.
Iron chelation is particularly difficult, as the treatment is not that pleasant to take, there is often no immediate feedback that it’s working (except urine discoloration with Ferriprox (deferiprone) and Desferal), and if you don’t take your chelation you may not become unwell for quite a few years. Thalassemia is fairly unique in this regard, and makes adherence even more challenging. Your healthcare team knows this and is sensitive to the challenges it poses to their patients.
More practical advice includes: peer support, journal keeping, alarm setting (on cellphone, etc.), use of smartphone Apps, utilizing the clinic resources available, such as a social worker or psychologist. Finally, is there a life event that can help you set a goal? For example, do you hope to marry in the next year or want to start a family? Is there a recent trigger to improve compliance, e.g. have you had a borderline blood glucose result and want to prevent diabetes from developing? These are often good incentives to reaching a chelation target. Make sure you are part of the conversation around what iron levels you would like to see, and explain why.
Remember no one is 100% adherent all the time, so don’t worry about trying to be perfect! (Chelation adherence averages 60-90% depending on the drug being studied.)