ICD-10 and other coding for SDS:
All you need to know
What is ICD or an ICD-10 code?
"ICD" is short for the "International Statistical Classification of Diseases and Related Health Problems." Globally, it's the go-to classification system in healthcare, used by government bodies and researchers for monitoring and reporting various health issues, from diseases to injuries. Overseen by the World Health Organization (WHO), the ICD system has a history that stretches back to 1893, having evolved through 11 major updates since then. While the US and another 150 countries utilize ICD-10, the WHO has released the next revision, ICD-11, in 2022. However, each country transitions to new ICD editions at its own pace, sometimes taking years for full integration. Notably, although WHO finalized the ICD-10 in 1992, the US only adopted it in 2015. We anticipate that the US will stay on ICD-10 for many more years to come, as there is a huge administrative burden with the transition and no fixed deadline for its adoption.
Up until now, SDS did not have its own code. SDS was basically invisible to the healthcare system...as if it didn't exist. As announced in this press release, this will soon change. Through a two-year effort by the SDS Alliance and its partners, the new ICD-10 code for SDS (D61.02) will go live in the US on October 1st, 2023.
Whenever someone goes to the doctor with an issue, the provider's team will enter their symptoms and diagnoses into the ICD coding system that precisely - or as precisely as possible - defines what is wrong. Insurance companies use these codes to help determine coverage for interventions provided. The codes also make it into large databases - but without personal patient information such as their name or contact information to protect their privacy. These large databases allow researchers to search the data to answer various health-related research questions.
Why do we need a code for SDS?
To understand the importance of having a unique and specific code for SDS, and since it's summer, let’s think through an example.
Let’s imagine you have SDS. It is a beautiful Saturday afternoon on a warm summer day, and you are enjoying an outdoor BBQ party with your friends and family. You apply your favorite mosquito repellent and are wearing long sleeves, but a nasty mosquito gets you anyway. The next day, the bite starts to hurt, swell and feel hot. It is now Sunday, so you decide to go to the ER to get it checked out. You receive a thorough exam, workup, and blood tests. Sure enough, the medical team there determines that it is cellulitis and that you need antibiotics. You go home with your doctor’s orders and make a full recovery within a few days. The staff at the hospital enter several codes into your medical record for billing purposes, such as a code for the ER visit, CBC with differential, blood cultures, blood pressure monitoring, neutropenia, cellulitis, and antibiotics. But no code for SDS - the root cause of the issues -, as there was none available.
Now let’s imagine a researcher who wants to perform a scientific review of how often SDS patients visit the ER or how often they get cellulitis from mosquito bites. Without an ICD-10 code specific to SDS, the researcher can only search the databases for the symptoms and interventions as they were coded, such as cellulitis, neutropenia, CBD with diff, and antibiotics, but not the root cause of these issues (which is SDS). None of these codes, nor their combination or clever filters, are specific enough to pinpoint an SDS patient. Not even close. We have tried it ourselves and even consulted with a biotech company to try to find meaningful data and make it work. So the researcher cannot answer any such research question related to SDS, without an SDS-specific code.
Now imagine how this will play out in the near future once the ICD-10 code for SDS (D61.02) goes into effect on October 1st, 2023, and gets wide-ranging use in the healthcare setting. More on this below. The researcher will easily be able to find data for their research on things like: How often do SDS patients use the ER? How often do they get cellulitis from mosquito bites? How were they treated? What was the outcome? Admission rate? Associated conditions or correlations with other symptoms? Where were they seen? And so on.
The ICD-code resource by the Everylife Foundation further explains:
“As described by the WHO, maintenance and use of this standard classification systems allows for:
Easy storage, retrieval and analysis of health information for evidenced-based decision-making
Sharing and comparing health information between hospitals, regions, settings and countries
Data comparisons in the same location across different time periods.
Consider the power of understanding which types of medical specialties are diagnosing a particular rare disease or having the ability to track changes in patients’ health outcomes after a new therapy is approved for their disease. A payer’s medical policy decisions for a new therapy might be based on an assessment of how many individuals covered by their plans have a particular diagnostic code in their record. These are the types of queries made possible through ICD codes. They are also the types of assessments that can be distorted by imprecise or out-of-date codes.”
And that is why we need YOUR help! Please read on.
What can YOU do to help?
To achieve the benefits described above, it is critical that doctors, other healthcare providers, and their teams use the SDS code (D61.02) every time they see an SDS patient. We have developed a tool kit (below) to help you spread the word.
Patients can help by telling all their healthcare providers about the new code, and encouraging other SDS families to do the same. We developed a toolbox to make it easy:
Share this webpage (www.sdsalliance.org/icd) with all healthcare providers who encounter SDS patients, or print this flyer and hand it to them. This includes the patient's primary care doctors, specialists, and other important healthcare providers such as nutritionists, PT, OT, SLPs.
Order your FREE promo toolkit including sticky notes (here) and hand them to all your healthcare providers at all upcoming appointments.
Share this information with all US SDS families, for example, by sharing our social media posts and this webpage.
Ask your healthcare providers to spread the word to their colleagues, too.
Fill out this emergency form (coming soon) and carry it with you in case of emergency. It is designed to help you all relevant information about the patient, plus the new code.
Healthcare providers can help by:
using the code for their SDS patients at every healthcare encounter
sharing this information with their networks
using the sticky notes available free of charge (here) to notify their coding team
How did the SDS Alliance and its collaborators make this happen?
First, it is important to acknowledge that given the importance of ICD codes, the process for updating codes – by design – is evidence-based, deliberative and thorough. Those who oversee the coding system must balance the needs of individuals and organizations seeking to revise codes with the integrity of the coding system as a whole.
The path to obtaining a new code for a previously unclassified disorder involves a long and challenging process.
In the US, the CDC’s ICD-10 Coordination and Maintenance Committee selects proposals for live presentations either at its headquarters in Baltimore or virtually twice a year. In December 2021, the SDS Alliance, aided by Medical Advisory Board member and NICER consortium director Dr. Kelly Walkovich, University of Michigan, C.S. Mott Children's Hospital, along with collaborator Dr. Alison Bertuch, Texas Children's Hospital and Baylor College of Medicine, submitted a proposal for a new code. Their proposal was then selected for presentation at the March 2022 meeting. During the public comment period that followed, major organizations such as the NIH iBMF study, the American Society of Hematology, the Leukemia and Lymphoma Society, AA/MDS, and The American Society of Pediatric Hematology/Oncology, submitted letters of support. The new code was officially accepted in July 2023 and will be active starting October 1st, 2023.
The SDS Alliance credits Dr. Terry Jo Bichell from COMBINEDBrain for her guidance and support throughout the effort.
More information on our partners and collaborators and the impact of the ICD-10 code are available in this press release.
What does the letter and number of the code mean?
The ICD-10-CM is highly structured and specific codes must conform to tightly managed classification conventions and go through a rigorous system to make any changes or additions.
The diagnosis coding system itself is divided into two parts:
Alphabetic Index: An alphabetical list of terms and their corresponding code. In the case of SDS, the code is under Chapter 3, Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89). This is where the D in D61.02 comes from.
Tabular List: A structured list of codes divided into chapters based on a body system or condition that contain categories, subcategories and codes that use a combination of letters and numerals to create unique identifiers. The ICD-10-CM uses an indented format for ease of reference.
Categories: Three characters – a letter followed by two numerals. If there is no further subdivision of a category, it is equivalent to a code. SDS got more. For SDS, it is Category: Aplastic and other anemias and other bone marrow failure syndromes (D60-D64). This is where D61 in D61.02 comes from.
Subcategories: The category (a letter plus two numerals), followed by a decimal point and then a subcategory (letter(s) or numeral(s)) for a total of either 4 or 5 characters. For SDS, these are the last two numbers or .02 in D61.02 to define the complete code.
Codes: May be a total of 3 to 7 characters. The 6th and 7th characters are generally used to provide additional context, for instance, with regard to timing or treatment status (controlled or uncontrolled), and are not used for SDS. As you know by now, the complete, unique, and specific code for SDS is D61.02.
Frequently Asked Questions (FAQ)
Does the new ICD-10 code for SDS (D61.02) replace all other codes my provider used in the past?
No! The new diagnostic code is to be used in addition to all the other codes your provider needs to use in order to describe the current issues they are treating to get your insurance to cover the cost. The new code for SDS indicates the underlying cause. In the future, it may help you get coverage for services by letting the insurance company know that it makes sense to cover specific services due to SDS, whereas otherwise, they would not. For example, because SDS is known to be a genetic disorder, insurance companies may be more likely to cover genetic testing if there is a clear indication, such as an SDS diagnosis (based on symptoms alone).
How is SDS defined for the ICD-10 code?
The ICD-10 code system does not include a set of symptoms or diagnostic criteria. It is simply a code to reflect in the medical record system that a patient is diagnosed with SDS. Making a diagnosis is under the treating physician's or specialists' purview, usually based upon published diagnostic guidelines, such as those listed on our publications and guidelines page.
Where can I learn more?
Learn more about ICD-10 codes at the ICD-code resource by the Everylife Foundation